Category Archive Autoimmune and Rare Diseases A – Z

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Transverse myelitis is a demyelinating and immune-mediated inflammatory disorder of the spinal cord, the part of the central nervous system that sends impulses from the brain to nerves in the body. This inflammation can cause damage to the ensheathing nerve cell fiber myelin, with resultant neurological dysfunction including weakness, sensory impairments, and autonomic problems including the bowel and bladder.^{[rx, rx, rx]} The spinal cord also carries sensory information back to the brain. The term myelitis refers to inflammation of the spinal cord; transverse refers to the pattern of changes in sensation—there is often a band-like sensation across the trunk of the body, with sensory changes below. Symptoms may develop suddenly (over a period of hours) or over days or weeks and include:

• pain
• sensory problems
• weakness in the legs and possibly arms, and
• bladder and bowel problems.

Transverse myelitis can affect people of any age, gender, or race. It does not appear to be genetic or run in families. The disorder typically occurs between ages 10 and 19 years and 30 and 39 years.

Although some people recover from transverse myelitis with minor or no residual problems, the healing process may take months to years. Most people with transverse myelitis have at least partial recovery, with most recovery taking place within the first 3 months after the attack. Others may suffer permanent impairments that affect their ability to perform ordinary tasks of daily living. Some individuals will have only one episode of transverse myelitis; other individuals may have a recurrence, especially if an underlying illness caused the disorder. There is no cure for transverse myelitis, but there are treatments to prevent or minimize permanent neurological deficits.

What causes transverse myelitis?

The exact cause of transverse myelitis and extensive damage to the bundles

of nerve fibers of the spinal cord is unknown in many cases. Cases in which a cause cannot be identified are called idiopathic. Viral, bacterial, and fungal infections affecting the spinal cord may cause the disorder.

A number of conditions appear to cause transverse myelitis, including:

• Immune system disorders appear to play an important role in causing damage to the spinal cord. Such disorders are:
  • an aquaporin-4 autoantibody associated neuromyelitis optic. Neuromyelitis Optica is a disorder that affects the eye nerves and spinal cord. Aquaporin-4 is a channel on the cell membrane that lets water enter the cell and helps maintain the chemical balance for processes to take place within the central nervous system. An antibody is a protein that binds to foreign substances that can attack the host organism.
  • multiple sclerosis, a disorder in which immune system cells that normally protect us from viruses, bacteria, and unhealthy cells mistakenly attack the protective coating of myelin in the brain, optic nerves, and spinal cord
  • the post-infectious or post-vaccine autoimmune phenomenon, in which the body’s immune system mistakenly attacks the body’s own tissue while responding to the infection or, less commonly, a vaccine
  • an abnormal immune response to underlying cancer that damages the nervous system; or
  • other antibody-mediated conditions that are still being discovered.
• Viral infections including herpes viruses such as varicella-zoster (the virus that causes chickenpox and shingles), herpes simplex, cytomegalovirus, and Epstein-Barr; flaviviruses such as West Nile and Zika; influenza, echovirus, hepatitis B, mumps, measles, and rubella. It is often difficult to know whether a direct viral infection or a post-infectious response causes transverse myelitis.
• Bacterial infections such as syphilis, tuberculosis, actinomyces, pertussis, tetanus, diphtheria, and Lyme disease. Bacterial skin infections, middle-ear infections, campylobacter jejuni gastroenteritis, and mycoplasma bacterial pneumonia have also been associated with the condition.
• Fungal infections in the spinal cord, including aspergillus, Blastomyces, coccidioides, and cryptococcus.
• Parasites, including toxoplasmosis, cysticercosis, schistosomiasis, and angtiostrongyloides.
• Other inflammatory disorders that can affect the spinal cord, such as sarcoidosis, systemic lupus erythematosus, Sjogren’s syndrome, mixed connective tissue disease, scleroderma, and Bechet’s syndrome.
• Vascular disorders such as arteriovenous malformation, dural arterial-venous fistula, intra-spinal cavernous malformations, or disk embolism.

or

• a.Multiple sclerosis
• b.NMO
• c.ADEM

• a.SLE
• b.SS
• c.Antiphospholipid syndrome
• d.Behçet disease
• e.Vogt-Koyanagi Harada disease
• f.Ankylosing spondylitis
• g.Mixed connective tissue disease
• h.Others: systemic sclerosis, anti-Jo-1 antibody, urticarial vasculitis, psoriatic arthritis, perinuclear ANCA systemic vasculitis, graft-versus-host disease, common variable immunodeficiency, celiac disease

• a.Viral: hepatitis A, hepatitis B, hepatitis C, hepatitis E, measles, mumps, rubella, varicella-zoster, Epstein-Barr, cytomegalovirus, herpes simplex, influenza A/B, lymphocytic choriomeningitis virus, chikungunya, Hanta virus, HIV, human T-cell lymphotropic virus, human herpesvirus 6, Japanese encephalitis, Murray Valley encephalitis, St. Louis encephalitis, tick-borne encephalitis, vaccnia. Rocky Mountain spotted fever, dengue virus, enterovirus 71, coxsackievirus A and B, West Nile virus, parvovirus B19, human corona virus, and echovirus
• b.Bacterial: Mycoplasma pneumoniae, Campylobacter jejuni, Borrelia burgdorferi, Acinetobacter baumanii, Coxiella burnetii, Bartonella henselae, Chlamydia psittaci, Leptospira, Chlamydia pneumoniae. Legionella pneumonia, Orientia tsutsugamushi (scrub typhus). Salmonella paratyphi B, Mycobacterium tuberculosis, Treponema pallidum, Brucellosis melitensis, and groups A and B strep to cocci
• c.Fungal: Actinomyces, Blastomyces, Coccidioides, Aspergillus, Cryptococcus, and Cladophialophora bantiana
• d.Parasitic: Toxocara species. Schistosoma species, Gnasthostoma spinigerum, Echinococcus granulosus, Taenia solium, Toxoplasma gondii, Acanthamoeba species, Paragonimus westermani, and Trypanosoma brucei

• a.Anti-Ri (ANNA-2) antibody
• b.CRMP-5-lgG antibody
• c.Anti-amphiphysin IgG antibody
• d.Anti-GAD65 antibody
• e.NMDAR antibody

• a.Tumor necrosis factor-alpha inhibitors

In some people, transverse myelitis represents the first symptom of an autoimmune or immune-mediated disease such as multiple sclerosis or neuromyelitis optica.“Partial” myelitis—affecting only a portion of the cord cross-section—is more characteristic of multiple sclerosis. Neuromyelitis Optica is much more likely as an underlying condition when the myelitis is “complete” (causing severe paralysis and numbness on both sides of the spinal cord). Myelitis attacks with neuromyelitis optica spectrum disorder (NMOSD) tend to be more severe and are associated with less recovery than attacks with multiple sclerosis.

What are the symptoms of transverse myelitis?

Transverse myelitis may be either acute(developing over hours to several days) or subacute(usually developing over one to four weeks).

The segment of the spinal cord at which the damage occurs determines which parts of the body are affected. Damage at one segment will affect function at that level and below. In individuals with transverse myelitis, myelin damage most often occurs in nerves in the upper back.

Four classic features of transverse myelitis are:

• Weakness of the legs and arms. People with transverse myelitis may have weakness in the legs that progresses rapidly. If the myelitis affects the upper spinal cord it affects the arms as well. Individuals may develop paraparesis(partial paralysis of the legs) that may progress to paraplegia(complete paralysis of the legs), requiring the person to use a wheelchair.
• Pain. Initial symptoms usually include lower back pain or sharp, shooting sensations that radiate down the legs or arms or around the torso.
• Sensory alterations. Transverse myelitis can cause paresthesias(abnormal sensations such as burning, tickling, pricking, numbness, coldness, or tingling) in the legs and sensory loss. Abnormal sensations in the torso and genital region are common.
• Bowel and bladder dysfunction. Common symptoms include an increased frequency or urge to use the toilet, incontinence, and constipation.

Many individuals also report experiencing muscle spasms, a general feeling of discomfort, headache, fever, and loss of appetite, while some people experience respiratory problems. Other symptoms may include sexual dysfunction and depression and anxiety caused by lifestyle changes, stress, and chronic pain.

or

Symptoms of transverse myelitis may develop within a few hours or days. Or, they may develop over 1 to 4 weeks. Symptoms can quickly become severe. Symptoms tend to occur at or below the damaged area of the spinal cord. Both sides of the body are often affected, but sometimes only one side is affected. Symptoms include:

Abnormal sensations

• Numbness
• Pricking
• Tingling
• Coldness
• Burning
• Sensitivity to touch or temperature

Bowel and bladder symptoms

• Constipation
• Frequent need to urinate
• Difficulty holding urine
• Urine leakage (incontinence)

Pain

• Sharp or blunt
• May start in your lower back
• May shoot down your arms and legs or wrap around your trunk or chest

Muscle weakness

• Loss of balance
• Difficulty walking (stumbling or dragging your feet)
• Partial loss of function, which may develop into paralysis

Sexual dysfunction

• Difficulty having an orgasm (men and women)
• Erectile dysfunction in men

Other symptoms can include loss of appetite, fever, and respiratory problems. Depression and anxiety can occur as a result of dealing with chronic pain and illness.

How is transverse myelitis diagnosed?

Physicians diagnose transverse myelitis by taking a medical history and performing a thorough neurological examination. Tests that can indicate a diagnosis of transverse myelitis and rule out or evaluate underlying causes include:

• Magnetic resonance imaging(MRI) produces a cross-sectional view or three-dimensional image of tissues, including the brain and spinal cord. A spinal MRI will almost always confirm the presence of a lesion within the spinal cord, whereas a brain MRI may provide clues to other underlying causes, especially MS. In some instances, computed tomography (CT) may be used to detect inflammation.
• Blood tests may be performed to rule out various disorders, including HIV infection and vitamin B12 deficiency. Blood is tested for the presence of autoantibodies (anti- aquaporin-4, anti-myelin oligodendrocyte) and antibodies associated with cancer (paraneoplastic antibodies). The presence of autoantibodies (proteins produced by cells of the immune system) is linked to autoimmune disorders and point to a definite cause of transverse myelitis.
• Lumbar puncture and spinal fluid analysis (also called a spinal tap) can identify more protein than usual in some people with transverse myelitis and an increased number of white blood cells (leukocytes) that help the body fight infections.

If none of these tests suggests a specific cause, the person is presumed to have idiopathic transverse myelitis.

How is transverse myelitis treated?

Non-Pharmacological

Common neurological deficits resulting from transverse myelitis include incontinence, chronic pain, and severe weakness, spasticity, or paralysis. In some cases, these may be permanent. Individuals with lasting or permanent neurological defects from transverse myelitis typically consult with a range of rehabilitation specialists, which may include physiatrists, physical therapists, occupational therapists, vocational therapists, and mental health care professionals.

• Physical therapy – can help retain muscle strength and flexibility, improve coordination, reduce spasticity, regain greater control over bladder and bowel function, and increase joint movement. Individuals are also taught to use assistive devices such as wheelchairs, canes, or braces.
• Occupational therapy – teaches people new ways to maintain or rebuild their independence by participating in meaningful, self-directed, everyday tasks such as bathing, dressing, preparing meals, and house cleaning.
• Vocational therapy – involves offering instructions to help people develop and promote work skills, identify potential employers, and assist in job searches. Vocational therapists act as mediators between employees and employers to secure reasonable workplace accommodations.
• Psychotherapy – for people living with permanent includes strategies and tools to deal with stress and a wide range of emotions and behaviors.

Medication

Treatments are designed to address infections that may cause the disorder, reduce spinal cord inflammation, and manage and alleviate symptoms.

Initial treatments and management of the complications of transverse myelitis include:

• Intravenous corticosteroid drugs – may decrease swelling and inflammation in the spine and reduce immune system activity. Such drugs may include methylprednisolone or dexamethasone. These medications may also be given to reduce subsequent attacks of transverse myelitis in individuals with underlying disorders.
• Plasma exchange therapy – (plasmapheresis) may be used for people who don’t respond well to intravenous steroids. Plasmapheresis is a procedure that reduces immune system activity by removing plasma (the fluid in which blood cells and antibodies are suspended) and replacing it with special fluids, thus removing the antibodies and other proteins thought to be causing the inflammatory reaction.
• Intravenous immunoglobulin (IVIG) – is a treatment thought to reset the immune system. IVIG is a highly concentrated injection of antibodies pooled from many healthy donors that bind to the antibodies that may cause the disorder and remove them from circulation.
• Pain medicines – that can lessen muscle pain include acetaminophen, ibuprofen, and naproxen. Nerve pain may be treated with certain antidepressant drugs (such as duloxetine), muscle relaxants (such as baclofen, tizanidine, or cyclobenzaprine), and anticonvulsant drugs (such as gabapentin or pregabalin).
• Antiviral medications – may help individuals who have a viral infection of the spinal cord.
• Medications can treat other symptoms and complications – including incontinence, painful muscle contractions called tonic spasms, stiffness, sexual dysfunction, and depression.

Following initial therapy, it is a critical part to keep the person’s body functioning during the recovery period. This may require placing the person on a respirator in the uncommon scenario where breathing is significantly affected.

Prevention of future transverse myelitis episodes

Multiple sclerosis and neuromyelitis optica typically require long-term treatment to modify the immune system response. Treatment of MS with immunomodulatory or immunosuppressant medications such as alemtuzumab, dimethyl fumarate, fingolimod, glatiramer acetate, interferon-beta, natalizumab, or teriflunomide may be needed.

Immunosuppressant treatments are used for neuromyelitis optica spectrum disorder and recurrent episodes of transverse myelitis that are not caused by multiple sclerosis. They are aimed at preventing future myelitis attacks (or attacks at other sites) and include steroid-sparing drugs such as mycophenolate mofetil, azathioprine, and rituximab.

What research is being done?

The mission of is to seek fundamental knowledge about the brain and nervous system and to use that knowledge to reduce the burden of neurological disease. NINDS is a component of the National Institutes of Health, the leading supporter of biomedical research in the world.

NINDS researchers are working to better understand how the immune system destroys or attacks the nerve-insulating substance called myelin in autoimmune diseases or disorders. Other work focuses on strategies to repair demyelinated spinal cords, including approaches using cell transplantation. This research may lead to a greater understanding of the mechanisms responsible for damaging myelin and may ultimately provide a means to prevent and treat transverse myelitis.

Glial cell studies. Glia, or neuroglia, are non-neuronal cells (they do not provide electrical impulses) in the nervous system that form myelin and provide support and protection for neurons. Oligodendrocyte progenitor cells (OPCs) are stem cells that generate myelin-producing oligodendrocytes, a type of glial cell. NINDS-funded scientists are studying cellular mechanisms that control the generation and maturation of OPCs to allow remyelination, which could be an effective therapy for transverse myelitis and spinal cord injury. Other NINDS-funded investigators are focusing on mechanisms and interventions designed to increase oligodendrocyte proliferation and remyelination after spinal cord injury.

Astrocytes are another type of glial cell. The aquaporin-4 IgG antibody binds to astrocytes, which has led to an increased interest in its role in transverse myelitis of neuromyelitis optica spectrum disorder (NMOSD). The antibody appears to cause myelitis in NMOSD by activating other components of the immune system, resulting in injury to the spinal cord. Many studies are trying to better understand the role of astrocytes in autoimmune diseases.

Genetic studies. NINDS-funded scientists hope to develop a better understanding of the molecular control of central nervous system myelination and remyelination by studying the theBrg1(Brahma-related) gene that appears to be involved in oligodendrocyte myelination. The long-term objective of this research is to develop drugs that modulate the activity ofBrg1and other genes to promote myelination and remyelination.

Animal models. NINDS funds research using animal models of spinal cord injury aimed at replacing or regenerating spinal cord nerve cells. The ultimate goals of these studies are to develop interventions for the regeneration or remyelination of spared nerve fibers in humans and to restore function to paralyzed individuals.

Neuroimaging with MRI. Research funded by aims to develop and implement new MRI techniques to quantitatively assess the relationship between spinal cord pathology and neurological dysfunction in MS. This new approach may assess changes in lesions and myelin in MS and possibly transverse myelitis. Other NIH-funded researchers plan to develop MRI methodologies to non-invasively detect and characterize networks to identify the extent of injury to the spinal cord and to monitor the progression of recovery after injury. These techniques may aid in earlier detection of transverse myelitis and other neurological disorders such as MS.

Brain-machine interfaces and prosthetic devices. Scientists are developing brain-machine interfaces and neural prostheses to help people with spinal cord damage regain functions by bypassing the injury site. These sophisticated electrical and mechanical devices connect with the nervous system to supplement or replace lost motor and sensory function.

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Resting tremor occurs when the muscle is relaxed, such as when the hands are resting on the lap.  With this disorder, a person’s hands, arms, or legs may shake even when they are at rest.  Often, the tremor only affects the hand or fingers.  This type of tremor is often seen in people with Parkinson’s disease and is called a “pill-rolling” tremor because the circular finger and hand movements resemble the rolling of small objects or pills in the hand.

Causes of Resting Tremor

Generally, tremor is caused by a problem in the deep parts of the brain that control movements.  Most types of tremors have no known cause, although there are some forms that appear to be inherited and run in families.

Tremor can occur on its own or be a symptom associated with a number of neurological disorders, including

• Multiple sclerosis
• Stroke
• Traumatic brain injury
• Neurodegenerative diseases that affect parts of the brain (e.g.,  Parkinson’s disease).
• Neurologic disorders, including multiple sclerosis, Parkinson’s disease, stroke, and traumatic brain injury
• Certain medicines, such as asthma medicines, amphetamines, caffeine, corticosteroids, and medicines used for certain psychiatric and neurological disorders
• Alcohol use disorder or alcohol withdrawal
• Mercury poisoning
• Hyperthyroidism (overactive thyroid)
• Liver or kidney failure
• Anxiety or panic

Some other known causes can include

• the use of certain medicines (particular asthma medication, amphetamines, caffeine, corticosteroids, and drugs used for certain psychiatric and neurological disorders)
• alcohol abuse or withdrawal
• mercury poisoning
• overactive thyroid
• liver or kidney failure
• anxiety or panic.

Symptoms of Resting Tremor

Symptoms of tremor may include

• A tremor is involuntary, rhythmic contractions of various muscles. Shaky legs syndrome causes feelings of “vibration”, unsteadiness or imbalance in the legs.
• A rhythmic shaking in the hands, arms, head, legs, or torso
• Balance and muscle coordination problem
• Spasticity and muscle spasm with wasting
• Shaky voice
• Difficulty writing or drawing
• Problems holding and controlling utensils, such as a spoon.
• Begin gradually, usually more prominently on one side of the body
• Worsen with movement
• Usually occur in the hands first, affecting one hand or both hands
• Can include a “yes-yes” or “no-no” motion of the head
• It May be aggravated by emotional stress, fatigue, caffeine, or temperature extremes
• Tremors that get worse during emotional stress
• Tremors that get worse when you move on purpose
• Tremors that lessen with rest
• Balance problems (in rare cases)
• Begin gradually, usually more prominently on one side of the body
• Worsen with movement

Essential tremor vs. Parkinson’s disease

Many people associate tremors with Parkinson’s disease, but the two conditions differ in key ways:

Timing of tremors. Essential tremor of the hands usually occurs when you use your hands. Tremors from Parkinson’s disease are most prominent when your hands are at your sides or resting in your lap.

Associated conditions. Essential tremor doesn’t cause other health problems, but Parkinson’s disease is associated with stooped posture, slow movement and shuffling gait. However, people with essential tremor sometimes develop other neurological signs and symptoms, such as an unsteady gait (ataxia).

Parts of body affected. Essential tremor mainly involves your hands, head and voice. Parkinson’s disease tremors usually start in your hands, and can affect your legs, chin and other parts of your body.

Signs

What we feel in both legs simultaneously is extreme straining, fatigue, unsteadiness, and a fear of falling. The muscles in our legs become hard, our ankles feel weak and our toes curl under as our legs fail to support us. We can stand for only a short period of time, in some cases only seconds. There is a feeling of panic to find a place to sit, or if possible, walk to gain some relief from our symptoms. Some tremors /shaky legs syndrome may be triggered by or become worse during times of stress or strong emotion, when an individual is physically exhausted, or when a person is in certain postures or makes certain movements.

Diagnosis of Resting Tremor

Medical history

During the physical evaluation, a doctor will assess the tremor based on:

• whether the tremor occurs when the muscles are at rest or inaction
• the location of the tremor on the body (and if it occurs on one or both sides of the body)
• the appearance of the tremor (tremor frequency and amplitude).

The doctor will also check other neurological findings such as impaired balance, speech abnormalities, or increased muscle stiffness.  Blood or urine tests can rule out metabolic causes such as thyroid malfunction and certain medications that can cause tremors/shaky legs syndrome.  These tests may also help to identify contributing causes such as drug interactions, chronic alcoholism, or other conditions or diseases.  Diagnostic imaging may help determine if the tremor is the result of damage to the brain.

Clinical observations

The diagnosis is based on a combination of a number of clinical observations and recognition of typical characteristics (phenomenology) that include, but are not necessarily limited to the following:

• Onset of the movements is abrupt/sudden.
• Movements are triggered by emotional or physical trauma, or by some conflict (marital, sexual, work-related).
• Movements are episodic or appear intermittent.
• There are spontaneous remissions of the movements.
• Movements disappear with distraction.
• Movements are suggestible, meaning they may disappear by making a suggestion. For example, suggesting that the application of a tuning fork to the body part affected may help relieve the movements.
• Underlying psychiatric disturbances (depression, anxiety) are present.
• There are multiple somatizations and undiagnosed conditions.
• There is a lack of emotional concern about the disorder (“la belle indifference”).
• There has been exposure to neurologic disorders during one’s occupation (e.g. nurse, physician) or while caring for someone with similar problems.
• Slurred speech, soft voice, gibberish, foreign accent
• Delayed and excessive startle (bizarre movements in response to sudden, unexpected noise or threatening movement)
• Presence of additional types of abnormal movements that are not known to be part of the primary or principal movement disorder pattern that the patient manifests
• Active resistance against passive movement
• Fixed posture

Treatment of Resting Tremor

Non-pharmacological

• Physical, speech-language, and occupational therapy – may help to control tremors and meet daily challenges caused by the tremor.  A physical therapist can help people improve their muscle control, functioning, and strength through coordination, balancing, and other exercises.  Some therapists recommend the use of weights, splints, other adaptive equipment, and special plates and utensils for eating.  Speech-language pathologists can evaluate and treat speech, language, communication, and swallowing disorders.  Occupational therapists can teach individuals new ways of performing activities of daily living that may be affected by tremors.
• Eliminating or reducing tremor-inducing substances such as caffeine and other medication – (such as stimulants) can help improve tremor. Though small amounts of alcohol can improve tremors for some people, tremors can become worse once the effects of the alcohol wear off.
• Interventional Therapy – For patients who fail pharmacologic treatment with the above drugs or are unable to tolerate the side effects, surgical options include deep brain stimulation (DBS), focused ultrasound, or radio-surgical gamma knife thalamotomy to treat persistently disabling limb tremor, and botulinum toxin injections to treat persistently disabling head or vocal cord tremor.
• Deep-brain stimulation – This is the most common surgical treatment for essential tremors. Most series report 70% to 90% hand tremor control. In deep-brain stimulation, electrical stimulation is delivered to the brain through an electrode implanted deep into the ventral intermediate nucleus (VIM) of the thalamus. This is typically done by implanting 4 electrodes in the VIM using stereotactic methods. Computerized programming of the pulse generator is most commonly done with a handheld device after the patient leaves the hospital to optimize the electrode montage, voltage, pulse frequency, and pulse width. Deep-brain stimulation can be done unilaterally or bilaterally depending on the patient’s symptoms. There is an increased risk of speech and balance difficulties with bilateral procedures. If the tremor significantly affects both hands, the dominant hand is targeted, bilateral procedures may be considered.
• Focused ultrasound – Approved by the FDA in 2016, magnetic resonance imaging-guided, high-intensity, focused ultrasound thalamotomy is an innovative method for the treatment of essential tremors. Although it is transcranial and does not require an incision, skull penetration, or an implanted device, it is an invasive therapy that produces a permanent thalamic lesion.
• Radio-surgical gamma knife thalamotomy – Gamma-knife thalamotomy fo­cuses high-energy gamma rays on the ventral intermediate resulting in the death of neurons. It is an unproven treatment that has not generally been adopted due to concerns about potential radiation side effects, including a theoretical, long-term risk of secondary tumor formation.
• Ultrasound Therapy – A new treatment for essential tremors uses magnetic resonance images to deliver focused ultrasound to create a lesion in tiny areas of the brain’s thalamus thought to be responsible for causing the tremors.  The treatment is approved only for those individuals with essential tremors who do not respond well to anticonvulsant or beta-blocking drugs.
• Biofeedback – is a mind-body technique that involves using visual or auditory feedback to teach people to recognize the physical signs and symptoms of stress and anxiety, such as increased heart rate, body temperature, and muscle tension.
• Relaxation techniques – can reduce stress symptoms and help you enjoy a better quality of life, especially if you have an illness. Explore relaxation techniques you can do by yourself.
• Learn to relax – Stress and anxiety tend to make tremors worse, and being relaxed may improve tremors. Although you can’t eliminate all stress from your life, you can change how you react to stressful situations using a range of relaxation techniques, such as massage or meditation.
• Noninvasive techniques – Include gait rehabilitation, visually guided techniques, tendon vibration, weighting extremities, positioning techniques, and manual techniques, all of which can be useful for the recovery of functional activities.[rx]
• Invasive techniques – Thalamic deep brain stimulation can alleviate the tremor in MS, providing better functional performance.[rx] Stereotactic radiosurgery thalamotomy at the nucleus ventralis intermedius with a median maximum dose of 140 Gy also provides good functional outcomes in patients with MS.[rx][rx] Radiofrequency thalamotomy had been successfully used in the past but has been replaced with the newer techniques of radiosurgery and deep brain stimulation as they had fewer adverse effects.
• Physical therapy – can help some patients control their tremors better, as can reducing the intake of substances such as caffeine, which can induce tremors. Many patients experience an increase in the severity of their tremors when they are stressed. Therefore, trying to reduce sources of anxiety and engaging in complementary therapies (such as yoga or aromatherapy) may help some patients.
• Speech and occupational therapy – may be useful not only in improving physical and psychological functioning, such as activities of daily living, but also to alter the abnormally learned pattern of movement – “motor reprograming.” Antidepressants and muscle relaxants may be also beneficial. Rarely, transcutaneous electrical stimulation applied to the area of spasm or involuntary movement may be helpful, analogous to the application of a tuning fork during clinic evaluation. Most importantly, however, the patient should try to understand which stress factors may be playing a role and seek the expertise of a psychologist experienced and skilled in stress management. The role of a psychiatrist is not to make the diagnosis but to provide insights into underlying psychological or psychiatric issues and to aid in the treatment of psychiatric issues such as depression or anxiety.
• Psychogenic movement Therapy –  It may be difficult to treat, especially if the patient is diagnosed late or is not accepting of the diagnosis. Indeed, patients with the best prognosis are those who initially accept the diagnosis and work with the movement disorder neurologist, psychologist, psychiatrist and physical, speech and occupational therapists in implementing a short-term and long-term therapeutic program. The diagnosis should be disclosed to patients in a manner that is empathetic and nonjudgmental.

For example, tremors due to thyroid hyperactivity will improve or even resolve (return to the normal state) with the treatment of thyroid malfunction.  Also, if the tremor is caused by medication, discontinuing the tremor-causing drug may reduce or eliminate this tremor.

Medical Therapy

The therapeutic approach to essential tremors many times follows a trial and error approach, and patients should be challenged by several medications if the first choice is ineffective or associated with debilitating adverse effects. Medical therapy can be divided into first, second, and third-line therapies.

First-line therapy – It is either approved by the FDA or supported by double-blinded, placebo-controlled studies that meet the criteria for the class I evidence. This class of medications includes propranolol and primidone. If both primidone and propranolol are not effective alone, combinations of both may provide relief in selected patients.

Second-line therapy – Second-line therapy is supported by double-blinded, placebo-controlled trials that do not meet other requirements for the class I evidence studies. This includes gabapentin, pregabalin, topiramate, benzodiazepines (clonazepam, alprazolam), beta-blockers (atenolol and metoprolol) and zonisamide.

Third-line therapy – These therapies are based on open-label studies or case series. Drugs in this class include nimodipine and clozapine.

Medication

• Beta-blocking drugs such as propranolol are normally used to treat high blood pressure but they also help treat essential tremors.  Propranolol can also be used in some people with other types of action tremors.  Other beta-blockers that may be used include atenolol, metoprolol, nadolol, and sotalol.
• Anti-seizure medications such as primidone can be effective in people with essential tremors who do not respond to beta-blockers.  Other medications that may be prescribed include gabapentin and topiramate.  However, it is important to note that some anti-seizure medications can cause tremors.
• Tranquilizers (also known as benzodiazepines) such as alprazolam and clonazepam may temporarily help some people with tremors.  However, their use is limited due to unwanted side effects that include sleepiness, poor concentration, and poor coordination.  This can affect the ability of people to perform daily activities such as driving, school, and work.  Also, when taken regularly, tranquilizers can cause physical dependence and when stopped abruptly can cause several withdrawal symptoms.
• Parkinson’s disease medications (levodopa, carbidopa) are used to treat tremors associated with Parkinson’s disease.
• Botulinum toxin – injections can treat almost all types of tremors.  It is especially useful for head tremor, which generally does not respond to medications.  Botulinum toxin is widely used to control dystonic tremors.  Although botulinum toxin injections can improve tremors for roughly three months at a time, they can also cause muscle weakness.  While this treatment is effective and usually well tolerated for head tremors, botulinum toxin treatment in the hands can cause weakness in the fingers.  It can cause a hoarse voice and difficulty swallowing when used to treat voice tremors.

Additional drug therapies that have been used to treat individuals with primary orthostatic tremors include primidone (Mysoline), chlordiazepoxide (Librium), pregabalin (Lyrica), pramipexole (Mirapex), phenobarbital, and valproic acid (Depakote). Drugs commonly used to treat people with Parkinson’s disease (levodopa or pramipexole) may also be prescribed for individuals with primary orthostatic tremors.

Surgery

When people do not respond to drug therapies or have a severe tremor that significantly impacts their daily life, a doctor may recommend surgical interventions such as deep brain stimulation (DBS) or very rarely, thalamotomy.  While DBS is usually well-tolerated, the most common side effects of tremor surgery include dysarthria (trouble speaking) and balance problems.

• Deep brain stimulation (DBS) – is the most common form of surgical treatment of tremors.  This method is preferred because it is effective, has low risk, and treats a broader range of symptoms than thalamotomy.  The treatment uses surgically implanted electrodes to send high-frequency electrical signals to the thalamus, the deep structure of the brain that coordinates and controls some involuntary movements.  A small pulse generating device placed under the skin in the upper chest (similar to a pacemaker) sends electrical stimuli to the brain and temporarily disables the tremor.  DBS is currently used to treat parkinsonian tremors, essential tremors, and dystonia.

• Thalamotomy – is a surgical procedure that involves the precise, permanent destruction of a tiny area in the thalamus.  Currently, surgery is replaced by radiofrequency ablation to treat severe tremors when deep brain surgery is contraindicated—meaning it is unwise as a treatment option or has undesirable side effects.  Radiofrequency ablation uses a radio wave to generate an electric current that heats up a nerve and disrupts its signaling ability for typically six or more months.  It is usually performed on only one side of the brain to improve tremors on the opposite side of the body.  Surgery on both sides is not recommended as it can cause problems with speech.
• Focused ultrasound thalamotomy – This non-invasive surgery involves using focused sound waves that travel through the skin and skull. The waves generate heat to destroy brain tissue in a specific area of the thalamus to stop a tremor. A surgeon uses magnetic resonance imaging to target the correct area of the brain and to be sure the sound waves are generating the exact amount of heat needed for the procedure. Focused ultrasound thalamotomy creates a lesion that can result in permanent changes to brain function. Some people have experienced the altered sensation, trouble with walking or difficulty with movement. However, most complications go away on their own or are mild enough that they don’t interfere with the quality of life.
•  Stereotactic surgical techniques – can be used to create a lesion in the ventral intermediate (VIM) nucleus of the thalamus.

Rehabilitation

Exercise is an important part of healthy living for everyone. For people with tremors, exercise is more than healthy it is a vital component to maintaining balance, mobility, and activities of daily living. Exercise and physical activity can improve many tremors symptoms. These benefits are supported by research.

The tremors show that people with tremors who start exercising earlier and a minimum of 2.5 hours a week, experience a slowed decline in quality of life compared to those who start later. Establishing early exercise habits is essential to overall disease management.

What Type of Exercise Should I Do?

To help manage the symptoms of tremors, be sure your exercise program includes a few key ingredients:

• Aerobic activity
• Strength training
• Balance, agility, and multitasking
• Flexibility

These elements are included in many types of exercise. Biking, running, Tai chi, yoga, Pilates, dance, weight training, non-contact boxing, qi gong, and more — all have positive effects on tremors symptoms.

There is no “exercise prescription” that is right for every person with tremors. The type of exercise you do depends on your symptoms and challenges. For sedentary people, just getting up and moving is beneficial. More active people can build up to the regular, vigorous activity. Many approaches work well to help maintain and improve mobility, flexibility, and balance to ease non-motor tremors symptoms such as depression or constipation.

Researchers in the study did not distinguish between what type of exercise participants did and determined that all types of exercise are beneficial. The most important thing is to do the exercise regularly. We suggest finding an exercise you enjoy and stick with it.

Challenges to Exercising

• People in the early stages of tremors tend to be just as strong and physically fit as healthy individuals of the same age.
• Disease progression can lead to the following physical change:
• Loss of joint flexibility, which can affect balance.
• Decreased muscle strength or deconditioning can affect walking and the ability to stand up from sitting.
• The decline in cardiovascular conditioning, which affects endurance.

Tips for Getting Started

• First, be safe. Before starting an exercise program, consult your neurologist and primary care doctor about concerns and recommendations.
• Ask your doctor or members in your support group to refer to a physical therapist (PT) who knows about tremors. Work together to identify your concerns and limitations. Target exercises to improve them. For most people, a structured exercise program will include aerobic exercise (such as brisk walking) and resistance training (using weights or bands).
• Purchase a pedometer (step-counter) and figure out how many steps you take on average each day, then build up from there. Many smartphones or smartwatches have a built-in pedometer feature or an application that can be downloaded.
• Exercise indoors and outdoors. Change your routine to stay interested and motivated.
• Again, most importantly pick an exercise you enjoy.

What research is being done?

The mission is to seek fundamental knowledge about the brain and nervous system and to use that knowledge to reduce the burden of neurological disease.  The NINDS is a component of the National Institutes of Health (NIH), the leading supporter of biomedical research in the world.

Researchers are working to better understand the underlying brain functions that cause tremors, identify the genetic factors that make individuals more susceptible to the disorder, and develop new and better treatment options.

• Brain functioning – It can be difficult to distinguish between movement disorders such as Parkinson’s disease and essential tremor. These debilitating movement disorders have different prognoses and can respond very differently to available therapies. NINDS researchers are working to identify structural and functional changes in the brain using non-invasive neuroimaging techniques to develop sensitive and specific markers for each of these diseases and then track how they change as each disease progresses. Other researchers are using functional magnetic resonance imaging technology to better understand normal and diseased brain circuit functions and associated motor behaviors.  Scientists hope to design therapies that can restore normal brain circuit function in diseases such as Parkinson’s disease and tremor.
• Genetics – Research has shown that essential tremors may have a strong genetic component affecting multiple generations of families.  NINDS researchers are building on previous genetics work to identify susceptibility genes for familial early-onset (before age 40) essential tremor.  Researchers are focusing on multigenerational, early-onset families to better detect linkages.

Additionally, NINDS scientists are researching the impact of genetic abnormalities on the development of essential tremors.  Previous research that has shown a link between essential tremor and possible genetic variants on chromosome 6 and 11; ongoing research is targeting the impact of other genetic variations in families.

Medications and other treatment methods

While drugs can be effective for some people, approximately 50 percent of individuals do not respond to medication.  In order to develop assistive and rehabilitative tremor-suppressing devices for people with essential tremors, researchers are exploring where and how to minimize or suppress tremors while still allowing for voluntary movements.

Many people with essential tremors respond to ethanol (alcohol); however, it is not clear why or how.  NINDS researchers are studying the impact of ethanol on tremors to determine the correct dosage amount and its physiological impact on the brain and whether other medications without the side effects of ethanol can be effective.

Other NIH researchers hope to identify the source of essential tremors, study the effects of currently available tremor-suppressant drugs on the brain, and develop more targeted and effective therapies.

FAQ

Please answer the following questions to participate in our certified Continuing Medical Education program. Only one answer is possible per question. Please select the answer that is most appropriate.

Question 1

Which of the following constellations of clinical findings is typical of tremor in patients with Parkinson’s disease?

1. bilateral postural tremor

2. unilateral rest tremor and diminished ipsilateral arm swing while walking

3. severe unilateral tremor while holding a cup or glass

4. tremor that only appears when the patient writes

5. postural tremor of both hands and ataxic gait

Question 2

A 25-year-old man has a mild postural tremor of both hands that improves when he drinks alcohol. His mother had the same condition. What can you advise him?

1. He should definitely be evaluated for possible early Parkinson’s disease.

2. He must get treatment now, as otherwise the condition could worsen.

3. If treatment is indicated, propranolol or primidone could be given.

4. Relaxation exercises and physiotherapy are effective treatment options.

5. Genetic testing is needed to confirm the diagnosis of essential tremor.

Question 3

A man who received the diagnosis of multiple sclerosis two years ago presents to you with the new onset of tremor. What constellation of clinical findings is typical of tremor due to multiple sclerosis?

1. rest tremor, only occasionally observable when the patient is excited

2. a tremor that appears sometimes on the left side, sometimes on the right

3. a tremor that is only present in the morning

4. a swaying, broad-based gait and an intention tremor

5. a postural tremor that is easily suppressed by voluntary effort

Question 4

A 55-year-old man with essential tremor says that he can no longer feed himself because of tremor, can dress himself only with great difficulty, and has not had legible handwriting for many years. Drug treatment as recommended in the relevant clinical guidelines brings only slight improvement. What can you advise the patient about the option of surgical treatment?

1. Deep brain stimulation (DBS) might help but is not available in Germany.

2. DBS is an experimental technique that is only performed in clinical trials.

3. DBS is indicated only to treat Parkinson’s disease and plays no role in the treatment of essential tremor.

4. DBS has a high chance of success in this situation; it is now established as a standard treatment for essential tremor.

5. DBS is no more effective than pharmacotherapy for this indication.

Question 5

What information is most important for the diagnostic classification of a tremor syndrome?

1. the clinical findings

2. brain magnetic resonance imaging (MRI) with fine cerebellar sections

3. nuclear-medical visualization of brain perfusion

4. ultrasonography of the basal ganglia

5. measurement of serum drug levels

Question 6

What findings indicate that tremor may be psychogenic?

1. no evidence of essential tremor or Parkinson’s disease on brain MRI

2. a longstanding marital conflict

3. a tremor of inconstant location that diminishes on distraction and is found to be irregular on tremor analysis

4. a clearly identifiable underlying psychological conflict

5. remission after psychotherapy

Question 7

When can tremor be treated surgically?

1. When the patient is unwilling to take drugs to treat tremor.

2. When the patient is under 50 years old.

3. When the tremor cannot be adequately suppressed by drugs and there is no contraindication to surgery.

4. When the patient is willing to see a neurosurgeon once a week so that brain stimulation can be performed.

5. When the patient is willing to assume the cost of weekly battery changes.

Question 8

What must be borne in mind with respect to drug treatment for various tremor syndromes?

1. That the treatment is based on the clinical findings and not on the underlying disease causing tremor.

2. That causally directed treatment is generally possible only for drug-induced tremors or tremors due to metabolic disturbance.

3. That parkinsonian tremor responds best to anticholinergic drugs and does not respond at all to the classic dopamine preparations.

4. That the cerebellar tremor of multiple sclerosis is treated in exactly the same way as essential tremor.

5. That essential tremor is usually medically intractable.

Question 9

What drugs can induce tremor?

1. lithium, valproic acid, cyclosporine A

2. carbamazepine, propranolol, Seroxat

3. aspirin, diclofenac, paracetamol

4. penicillin, erythromycin, cephalosporin

5. antilipid drugs, antidiabetic drugs

Question 10

What is the drug, or drug class, of first choice for the treatment of parkinsonian tremor?

1. dopaminergic drugs

2. propanolol

3. primidone

4. gabapentin

5. ondansetron

References

People Also Reading

Tremor – Causes, Symptoms, Diagnosis, Treatment Orthostatic Tremor – Causes, Symptoms, Diagnosis, Treatment Parkinsonian Tremor – Causes, Symptoms, Diagnosis, Treatment Physiologic Tremor – Causes, Symptoms, Treatment Cerebellar Tremor – Causes, Symptoms, Treatment Idiopathic Orthostatic Tremor – Causes, Symptoms, Treatment Primary Orthostatic Tremor – Causes, Symptoms, Treatment Enhanced Physiological Tremor – Causes, Symptoms, Treatment Functional Tremor – Causes, Symptoms, Treatment Psychogenic Tremor – Causes, Symptoms, Treatment Postural Tremor – Causes, Symptoms, Treatment Dystonic Tremor – Causes, Symptoms, Treatment

Cerebellar tremor is typically a slow, high-amplitude (easily visible) tremor of the extremities (e.g., arm, leg) that occurs at the end of a purposeful movement such as trying to press a button.  It is caused by damage to the cerebellum and its pathways to other brain regions resulting from a stroke or tumor.  Damage also may be caused by diseases such as multiple sclerosis or an inherited degenerative disorder such as ataxia (in which people lose muscle control in the arms and legs) and Fragile X syndrome (a disorder marked by a range of intellectual and developmental problems).  It can also result from chronic damage to the cerebellum due to alcoholism.

Cerebellar tremor is often used synonymously with intention tremor. However, several clinical types of action tremor are included in this category with intention tremor being the most common form. Intention tremor typically increases during the approach to a target. Action, kinetic, and titubation or stance tremors are usually regarded as being of cerebellar origin if other signs of cerebellar dysfunction are also present. According to the consensus statement of the Movement Disorder Society on tremor, cerebellar tremors can be diagnosed according to the following clinical signs: (1) pure or dominant intention tremor, either unilateral or bilateral; (2) tremor frequency usually less than 5 Hz; and (3) postural tremor present without rest tremor.

Causes of Cerebellar Tremor

Generally, tremor is caused by a problem in the deep parts of the brain that control movements.  Most types of tremors have no known cause, although there are some forms that appear to be inherited and run in families.

Tremor can occur on its own or be a symptom associated with a number of neurological disorders, including

• Multiple sclerosis
• Stroke
• Traumatic brain injury
• Neurodegenerative diseases that affect parts of the brain (e.g.,  Parkinson’s disease).
• Neurologic disorders, including multiple sclerosis, Parkinson’s disease, stroke, and traumatic brain injury
• Certain medicines, such as asthma medicines, amphetamines, caffeine, corticosteroids, and medicines used for certain psychiatric and neurological disorders
• Alcohol use disorder or alcohol withdrawal
• Mercury poisoning
• Hyperthyroidism (overactive thyroid)
• Liver or kidney failure
• Anxiety or panic

Some other known causes can include

• the use of certain medicines (particular asthma medication, amphetamines, caffeine, corticosteroids, and drugs used for certain psychiatric and neurological disorders)
• alcohol abuse or withdrawal
• mercury poisoning
• overactive thyroid
• liver or kidney failure
• anxiety or panic.

Symptoms of Cerebellar Tremor

Symptoms of tremor may include

• A tremor is involuntary, rhythmic contractions of various muscles. Shaky legs syndrome causes feelings of “vibration”, unsteadiness or imbalance in the legs.
• A rhythmic shaking in the hands, arms, head, legs, or torso
• Balance and muscle coordination problem
• Spasticity and muscle spasm with wasting
• Shaky voice
• Difficulty writing or drawing
• Problems holding and controlling utensils, such as a spoon.
• Begin gradually, usually more prominently on one side of the body
• Worsen with movement
• Usually occur in the hands first, affecting one hand or both hands
• Can include a “yes-yes” or “no-no” motion of the head
• It May be aggravated by emotional stress, fatigue, caffeine, or temperature extremes
• Tremors that get worse during emotional stress
• Tremors that get worse when you move on purpose
• Tremors that lessen with rest
• Balance problems (in rare cases)

Signs

What we feel in both legs simultaneously is extreme straining, fatigue, unsteadiness, and a fear of falling. The muscles in our legs become hard, our ankles feel weak and our toes curl under as our legs fail to support us. We can stand for only a short period of time, in some cases only seconds. There is a feeling of panic to find a place to sit, or if possible, walk to gain some relief from our symptoms. Some tremors /shaky legs syndrome may be triggered by or become worse during times of stress or strong emotion, when an individual is physically exhausted, or when a person is in certain postures or makes certain movements.

Diagnosis of Cerebellar Tremor

Medical history

During the physical evaluation, a doctor will assess the tremor based on:

• whether the tremor occurs when the muscles are at rest or inaction
• the location of the tremor on the body (and if it occurs on one or both sides of the body)
• the appearance of the tremor (tremor frequency and amplitude).

The doctor will also check other neurological findings such as impaired balance, speech abnormalities, or increased muscle stiffness.  Blood or urine tests can rule out metabolic causes such as thyroid malfunction and certain medications that can cause tremors/shaky legs syndrome.  These tests may also help to identify contributing causes such as drug interactions, chronic alcoholism, or other conditions or diseases.  Diagnostic imaging may help determine if the tremor is the result of damage to the brain.

Clinical observations

The diagnosis is based on a combination of a number of clinical observations and recognition of typical characteristics (phenomenology) that include, but are not necessarily limited to the following:

• Onset of the movements is abrupt/sudden.
• Movements are triggered by emotional or physical trauma, or by some conflict (marital, sexual, work-related).
• Movements are episodic or appear intermittent.
• There are spontaneous remissions of the movements.
• Movements disappear with distraction.
• Movements are suggestible, meaning they may disappear by making a suggestion. For example, suggesting that the application of a tuning fork to the body part affected may help relieve the movements.
• Underlying psychiatric disturbances (depression, anxiety) are present.
• There are multiple somatizations and undiagnosed conditions.
• There is a lack of emotional concern about the disorder (“la belle indifference”).
• There has been exposure to neurologic disorders during one’s occupation (e.g. nurse, physician) or while caring for someone with similar problems.
• Slurred speech, soft voice, gibberish, foreign accent
• Delayed and excessive startle (bizarre movements in response to sudden, unexpected noise or threatening movement)
• Presence of additional types of abnormal movements that are not known to be part of the primary or principal movement disorder pattern that the patient manifests
• Active resistance against passive movement
• Fixed posture

Treatment of Cerebellar Tremor

Non-pharmacological

• Physical, speech-language, and occupational therapy – may help to control tremors and meet daily challenges caused by the tremor.  A physical therapist can help people improve their muscle control, functioning, and strength through coordination, balancing, and other exercises.  Some therapists recommend the use of weights, splints, other adaptive equipment, and special plates and utensils for eating.  Speech-language pathologists can evaluate and treat speech, language, communication, and swallowing disorders.  Occupational therapists can teach individuals new ways of performing activities of daily living that may be affected by tremors.
• Eliminating or reducing tremor-inducing substances such as caffeine and other medication – (such as stimulants) can help improve tremor. Though small amounts of alcohol can improve tremors for some people, tremors can become worse once the effects of the alcohol wear off.
• Interventional Therapy – For patients who fail pharmacologic treatment with the above drugs or are unable to tolerate the side effects, surgical options include deep brain stimulation (DBS), focused ultrasound, or radio-surgical gamma knife thalamotomy to treat persistently disabling limb tremor, and botulinum toxin injections to treat persistently disabling head or vocal cord tremor.
• Deep-brain stimulation – This is the most common surgical treatment for essential tremors. Most series report 70% to 90% hand tremor control. In deep-brain stimulation, electrical stimulation is delivered to the brain through an electrode implanted deep into the ventral intermediate nucleus (VIM) of the thalamus. This is typically done by implanting 4 electrodes in the VIM using stereotactic methods. Computerized programming of the pulse generator is most commonly done with a handheld device after the patient leaves the hospital to optimize the electrode montage, voltage, pulse frequency, and pulse width. Deep-brain stimulation can be done unilaterally or bilaterally depending on the patient’s symptoms. There is an increased risk of speech and balance difficulties with bilateral procedures. If the tremor significantly affects both hands, the dominant hand is targeted, bilateral procedures may be considered.
• Focused ultrasound – Approved by the FDA in 2016, magnetic resonance imaging-guided, high-intensity, focused ultrasound thalamotomy is an innovative method for the treatment of essential tremors. Although it is transcranial and does not require an incision, skull penetration, or an implanted device, it is an invasive therapy that produces a permanent thalamic lesion.
• Radio-surgical gamma knife thalamotomy – Gamma-knife thalamotomy fo­cuses high-energy gamma rays on the ventral intermediate resulting in the death of neurons. It is an unproven treatment that has not generally been adopted due to concerns about potential radiation side effects, including a theoretical, long-term risk of secondary tumor formation.
• Ultrasound Therapy – A new treatment for essential tremors uses magnetic resonance images to deliver focused ultrasound to create a lesion in tiny areas of the brain’s thalamus thought to be responsible for causing the tremors.  The treatment is approved only for those individuals with essential tremors who do not respond well to anticonvulsant or beta-blocking drugs.
• Biofeedback – is a mind-body technique that involves using visual or auditory feedback to teach people to recognize the physical signs and symptoms of stress and anxiety, such as increased heart rate, body temperature, and muscle tension.
• Relaxation techniques – can reduce stress symptoms and help you enjoy a better quality of life, especially if you have an illness. Explore relaxation techniques you can do by yourself.
• Learn to relax – Stress and anxiety tend to make tremors worse, and being relaxed may improve tremors. Although you can’t eliminate all stress from your life, you can change how you react to stressful situations using a range of relaxation techniques, such as massage or meditation.
• Noninvasive techniques – Include gait rehabilitation, visually guided techniques, tendon vibration, weighting extremities, positioning techniques, and manual techniques, all of which can be useful for the recovery of functional activities.[rx]
• Invasive techniques – Thalamic deep brain stimulation can alleviate the tremor in MS, providing better functional performance.[rx] Stereotactic radiosurgery thalamotomy at the nucleus ventralis intermedius with a median maximum dose of 140 Gy also provides good functional outcomes in patients with MS.[rx][rx] Radiofrequency thalamotomy had been successfully used in the past but has been replaced with the newer techniques of radiosurgery and deep brain stimulation as they had fewer adverse effects.
• Physical therapy – can help some patients control their tremors better, as can reducing the intake of substances such as caffeine, which can induce tremors. Many patients experience an increase in the severity of their tremors when they are stressed. Therefore, trying to reduce sources of anxiety and engaging in complementary therapies (such as yoga or aromatherapy) may help some patients.
• Speech and occupational therapy – may be useful not only in improving physical and psychological functioning, such as activities of daily living, but also to alter the abnormally learned pattern of movement – “motor reprograming.” Antidepressants and muscle relaxants may be also beneficial. Rarely, transcutaneous electrical stimulation applied to the area of spasm or involuntary movement may be helpful, analogous to the application of a tuning fork during clinic evaluation. Most importantly, however, the patient should try to understand which stress factors may be playing a role and seek the expertise of a psychologist experienced and skilled in stress management. The role of a psychiatrist is not to make the diagnosis but to provide insights into underlying psychological or psychiatric issues and to aid in the treatment of psychiatric issues such as depression or anxiety.
• Psychogenic movement Therapy –  It may be difficult to treat, especially if the patient is diagnosed late or is not accepting of the diagnosis. Indeed, patients with the best prognosis are those who initially accept the diagnosis and work with the movement disorder neurologist, psychologist, psychiatrist and physical, speech and occupational therapists in implementing a short-term and long-term therapeutic program. The diagnosis should be disclosed to patients in a manner that is empathetic and nonjudgmental.

For example, tremors due to thyroid hyperactivity will improve or even resolve (return to the normal state) with the treatment of thyroid malfunction.  Also, if the tremor is caused by medication, discontinuing the tremor-causing drug may reduce or eliminate this tremor.

Medical Therapy

The therapeutic approach to essential tremors many times follows a trial and error approach, and patients should be challenged by several medications if the first choice is ineffective or associated with debilitating adverse effects. Medical therapy can be divided into first, second, and third-line therapies.

First-line therapy – It is either approved by the FDA or supported by double-blinded, placebo-controlled studies that meet the criteria for the class I evidence. This class of medications includes propranolol and primidone. If both primidone and propranolol are not effective alone, combinations of both may provide relief in selected patients.

Second-line therapy – Second-line therapy is supported by double-blinded, placebo-controlled trials that do not meet other requirements for the class I evidence studies. This includes gabapentin, pregabalin, topiramate, benzodiazepines (clonazepam, alprazolam), beta-blockers (atenolol and metoprolol) and zonisamide.

Third-line therapy – These therapies are based on open-label studies or case series. Drugs in this class include nimodipine and clozapine.

Medication

• Beta-blocking drugs such as propranolol are normally used to treat high blood pressure but they also help treat essential tremors.  Propranolol can also be used in some people with other types of action tremors.  Other beta-blockers that may be used include atenolol, metoprolol, nadolol, and sotalol.
• Anti-seizure medications such as primidone can be effective in people with essential tremors who do not respond to beta-blockers.  Other medications that may be prescribed include gabapentin and topiramate.  However, it is important to note that some anti-seizure medications can cause tremors.
• Tranquilizers (also known as benzodiazepines) such as alprazolam and clonazepam may temporarily help some people with tremors.  However, their use is limited due to unwanted side effects that include sleepiness, poor concentration, and poor coordination.  This can affect the ability of people to perform daily activities such as driving, school, and work.  Also, when taken regularly, tranquilizers can cause physical dependence and when stopped abruptly can cause several withdrawal symptoms.
• Parkinson’s disease medications (levodopa, carbidopa) are used to treat tremors associated with Parkinson’s disease.
• Botulinum toxin – injections can treat almost all types of tremors.  It is especially useful for head tremor, which generally does not respond to medications.  Botulinum toxin is widely used to control dystonic tremors.  Although botulinum toxin injections can improve tremors for roughly three months at a time, they can also cause muscle weakness.  While this treatment is effective and usually well tolerated for head tremors, botulinum toxin treatment in the hands can cause weakness in the fingers.  It can cause a hoarse voice and difficulty swallowing when used to treat voice tremors.

Additional drug therapies that have been used to treat individuals with primary orthostatic tremors include primidone (Mysoline), chlordiazepoxide (Librium), pregabalin (Lyrica), pramipexole (Mirapex), phenobarbital, and valproic acid (Depakote). Drugs commonly used to treat people with Parkinson’s disease (levodopa or pramipexole) may also be prescribed for individuals with primary orthostatic tremors.

Surgery

When people do not respond to drug therapies or have a severe tremor that significantly impacts their daily life, a doctor may recommend surgical interventions such as deep brain stimulation (DBS) or very rarely, thalamotomy.  While DBS is usually well-tolerated, the most common side effects of tremor surgery include dysarthria (trouble speaking) and balance problems.

• Deep brain stimulation (DBS) – is the most common form of surgical treatment of tremors.  This method is preferred because it is effective, has low risk, and treats a broader range of symptoms than thalamotomy.  The treatment uses surgically implanted electrodes to send high-frequency electrical signals to the thalamus, the deep structure of the brain that coordinates and controls some involuntary movements.  A small pulse generating device placed under the skin in the upper chest (similar to a pacemaker) sends electrical stimuli to the brain and temporarily disables the tremor.  DBS is currently used to treat parkinsonian tremors, essential tremors, and dystonia.

• Thalamotomy – is a surgical procedure that involves the precise, permanent destruction of a tiny area in the thalamus.  Currently, surgery is replaced by radiofrequency ablation to treat severe tremors when deep brain surgery is contraindicated—meaning it is unwise as a treatment option or has undesirable side effects.  Radiofrequency ablation uses a radio wave to generate an electric current that heats up a nerve and disrupts its signaling ability for typically six or more months.  It is usually performed on only one side of the brain to improve tremors on the opposite side of the body.  Surgery on both sides is not recommended as it can cause problems with speech.
•  Stereotactic surgical techniques – can be used to create a lesion in the ventral intermediate (VIM) nucleus of the thalamus.

Rehabilitation

Exercise is an important part of healthy living for everyone. For people with tremors, exercise is more than healthy it is a vital component to maintaining balance, mobility, and activities of daily living. Exercise and physical activity can improve many tremors symptoms. These benefits are supported by research.

The tremors show that people with tremors who start exercising earlier and a minimum of 2.5 hours a week, experience a slowed decline in quality of life compared to those who start later. Establishing early exercise habits is essential to overall disease management.

What Type of Exercise Should I Do?

To help manage the symptoms of tremors, be sure your exercise program includes a few key ingredients:

• Aerobic activity
• Strength training
• Balance, agility, and multitasking
• Flexibility

These elements are included in many types of exercise. Biking, running, Tai chi, yoga, Pilates, dance, weight training, non-contact boxing, qi gong, and more — all have positive effects on tremors symptoms.

There is no “exercise prescription” that is right for every person with tremors. The type of exercise you do depends on your symptoms and challenges. For sedentary people, just getting up and moving is beneficial. More active people can build up to the regular, vigorous activity. Many approaches work well to help maintain and improve mobility, flexibility, and balance to ease non-motor tremors symptoms such as depression or constipation.

Researchers in the study did not distinguish between what type of exercise participants did and determined that all types of exercise are beneficial. The most important thing is to do the exercise regularly. We suggest finding an exercise you enjoy and stick with it.

Challenges to Exercising

• People in the early stages of tremors tend to be just as strong and physically fit as healthy individuals of the same age.
• Disease progression can lead to the following physical change:
• Loss of joint flexibility, which can affect balance.
• Decreased muscle strength or deconditioning can affect walking and the ability to stand up from sitting.
• The decline in cardiovascular conditioning, which affects endurance.

Tips for Getting Started

• First, be safe. Before starting an exercise program, consult your neurologist and primary care doctor about concerns and recommendations.
• Ask your doctor or members in your support group to refer to a physical therapist (PT) who knows about tremors. Work together to identify your concerns and limitations. Target exercises to improve them. For most people, a structured exercise program will include aerobic exercise (such as brisk walking) and resistance training (using weights or bands).
• Purchase a pedometer (step-counter) and figure out how many steps you take on average each day, then build up from there. Many smartphones or smartwatches have a built-in pedometer feature or an application that can be downloaded.
• Exercise indoors and outdoors. Change your routine to stay interested and motivated.
• Again, most importantly pick an exercise you enjoy.

What research is being done?

The mission is to seek fundamental knowledge about the brain and nervous system and to use that knowledge to reduce the burden of neurological disease.  The NINDS is a component of the National Institutes of Health (NIH), the leading supporter of biomedical research in the world.

Researchers are working to better understand the underlying brain functions that cause tremors, identify the genetic factors that make individuals more susceptible to the disorder, and develop new and better treatment options.

• Brain functioning – It can be difficult to distinguish between movement disorders such as Parkinson’s disease and essential tremor. These debilitating movement disorders have different prognoses and can respond very differently to available therapies. NINDS researchers are working to identify structural and functional changes in the brain using non-invasive neuroimaging techniques to develop sensitive and specific markers for each of these diseases and then track how they change as each disease progresses. Other researchers are using functional magnetic resonance imaging technology to better understand normal and diseased brain circuit functions and associated motor behaviors.  Scientists hope to design therapies that can restore normal brain circuit function in diseases such as Parkinson’s disease and tremor.
• Genetics – Research has shown that essential tremors may have a strong genetic component affecting multiple generations of families.  NINDS researchers are building on previous genetics work to identify susceptibility genes for familial early-onset (before age 40) essential tremor.  Researchers are focusing on multigenerational, early-onset families to better detect linkages.

Additionally, NINDS scientists are researching the impact of genetic abnormalities on the development of essential tremors.  Previous research that has shown a link between essential tremor and possible genetic variants on chromosome 6 and 11; ongoing research is targeting the impact of other genetic variations in families.

Medications and other treatment methods

While drugs can be effective for some people, approximately 50 percent of individuals do not respond to medication.  In order to develop assistive and rehabilitative tremor-suppressing devices for people with essential tremors, researchers are exploring where and how to minimize or suppress tremors while still allowing for voluntary movements.

Many people with essential tremors respond to ethanol (alcohol); however, it is not clear why or how.  NINDS researchers are studying the impact of ethanol on tremors to determine the correct dosage amount and its physiological impact on the brain and whether other medications without the side effects of ethanol can be effective.

Other NIH researchers hope to identify the source of essential tremors, study the effects of currently available tremor-suppressant drugs on the brain, and develop more targeted and effective therapies.

FAQ

Please answer the following questions to participate in our certified Continuing Medical Education program. Only one answer is possible per question. Please select the answer that is most appropriate.

Question 1

Which of the following constellations of clinical findings is typical of tremor in patients with Parkinson’s disease?

1. bilateral postural tremor

2. unilateral rest tremor and diminished ipsilateral arm swing while walking

3. severe unilateral tremor while holding a cup or glass

4. tremor that only appears when the patient writes

5. postural tremor of both hands and ataxic gait

Question 2

A 25-year-old man has a mild postural tremor of both hands that improves when he drinks alcohol. His mother had the same condition. What can you advise him?

1. He should definitely be evaluated for possible early Parkinson’s disease.

2. He must get treatment now, as otherwise the condition could worsen.

3. If treatment is indicated, propranolol or primidone could be given.

4. Relaxation exercises and physiotherapy are effective treatment options.

5. Genetic testing is needed to confirm the diagnosis of essential tremor.

Question 3

A man who received the diagnosis of multiple sclerosis two years ago presents to you with the new onset of tremor. What constellation of clinical findings is typical of tremor due to multiple sclerosis?

1. rest tremor, only occasionally observable when the patient is excited

2. a tremor that appears sometimes on the left side, sometimes on the right

3. a tremor that is only present in the morning

4. a swaying, broad-based gait and an intention tremor

5. a postural tremor that is easily suppressed by voluntary effort

Question 4

A 55-year-old man with essential tremor says that he can no longer feed himself because of tremor, can dress himself only with great difficulty, and has not had legible handwriting for many years. Drug treatment as recommended in the relevant clinical guidelines brings only slight improvement. What can you advise the patient about the option of surgical treatment?

1. Deep brain stimulation (DBS) might help but is not available in Germany.

2. DBS is an experimental technique that is only performed in clinical trials.

3. DBS is indicated only to treat Parkinson’s disease and plays no role in the treatment of essential tremor.

4. DBS has a high chance of success in this situation; it is now established as a standard treatment for essential tremor.

5. DBS is no more effective than pharmacotherapy for this indication.

Question 5

What information is most important for the diagnostic classification of a tremor syndrome?

1. the clinical findings

2. brain magnetic resonance imaging (MRI) with fine cerebellar sections

3. nuclear-medical visualization of brain perfusion

4. ultrasonography of the basal ganglia

5. measurement of serum drug levels

Question 6

What findings indicate that tremor may be psychogenic?

1. no evidence of essential tremor or Parkinson’s disease on brain MRI

2. a longstanding marital conflict

3. a tremor of inconstant location that diminishes on distraction and is found to be irregular on tremor analysis

4. a clearly identifiable underlying psychological conflict

5. remission after psychotherapy

Question 7

When can tremor be treated surgically?

1. When the patient is unwilling to take drugs to treat tremor.

2. When the patient is under 50 years old.

3. When the tremor cannot be adequately suppressed by drugs and there is no contraindication to surgery.

4. When the patient is willing to see a neurosurgeon once a week so that brain stimulation can be performed.

5. When the patient is willing to assume the cost of weekly battery changes.

Question 8

What must be borne in mind with respect to drug treatment for various tremor syndromes?

1. That the treatment is based on the clinical findings and not on the underlying disease causing tremor.

2. That causally directed treatment is generally possible only for drug-induced tremors or tremors due to metabolic disturbance.

3. That parkinsonian tremor responds best to anticholinergic drugs and does not respond at all to the classic dopamine preparations.

4. That the cerebellar tremor of multiple sclerosis is treated in exactly the same way as essential tremor.

5. That essential tremor is usually medically intractable.

Question 9

What drugs can induce tremor?

1. lithium, valproic acid, cyclosporine A

2. carbamazepine, propranolol, Seroxat

3. aspirin, diclofenac, paracetamol

4. penicillin, erythromycin, cephalosporin

5. antilipid drugs, antidiabetic drugs

Question 10

What is the drug, or drug class, of first choice for the treatment of parkinsonian tremor?

1. dopaminergic drugs

2. propanolol

3. primidone

4. gabapentin

5. ondansetron

References

People Also Reading

Tremor – Causes, Symptoms, Diagnosis, Treatment Paraneoplastic Cerebellar Degeneration – Symptoms, Treatment Physiologic Tremor – Causes, Symptoms, Treatment Cerebellar Dysfunction – Causes, Symptoms, Treatment Resting Tremor – Causes, Symptoms, Diagnosis, Treatment Orthostatic Tremor – Causes, Symptoms, Diagnosis, Treatment Idiopathic Orthostatic Tremor – Causes, Symptoms, Treatment Primary Orthostatic Tremor – Causes, Symptoms, Treatment Enhanced Physiological Tremor – Causes, Symptoms, Treatment Functional Tremor – Causes, Symptoms, Treatment Psychogenic Tremor – Causes, Symptoms, Treatment Postural Tremor – Causes, Symptoms, Treatment

Physiologic tremor/ Physiologic tremor occurs in all healthy individuals.  It is rarely visible to the eye and typically involves a fine shaking of both of the hands and also the fingers.  It is not considered a disease but is a normal human phenomenon that is the result of physical properties in the body (for example, rhythmical activities such as heartbeat and muscle activation).

Physiologic tremor is a tremor or trembling of a limb or other body part. The usual frequency is about 10 times per second. It occurs in normal individuals, especially when they are stressed by anxiety or fatigue. It is therefore common in sports such as rock-climbing where it is known by names such as Elvis leg or sewing machine leg.^[rx] It may occur in an enhanced form as a pathological symptom of conditions such as hyperthyroidism or stimulants such as caffeine.^[rx]

Causes of Physiologic Tremor

Generally, tremor is caused by a problem in the deep parts of the brain that control movements.  Most types of tremors have no known cause, although there are some forms that appear to be inherited and run in families.

Tremor can occur on its own or be a symptom associated with a number of neurological disorders, including

• Multiple sclerosis
• Stroke
• Traumatic brain injury
• Neurodegenerative diseases that affect parts of the brain (e.g.,  Parkinson’s disease).
• Neurologic disorders, including multiple sclerosis, Parkinson’s disease, stroke, and traumatic brain injury
• Certain medicines, such as asthma medicines, amphetamines, caffeine, corticosteroids, and medicines used for certain psychiatric and neurological disorders
• Alcohol use disorder or alcohol withdrawal
• Mercury poisoning
• Hyperthyroidism (overactive thyroid)
• Liver or kidney failure
• Anxiety or panic

Some other known causes can include

• the use of certain medicines (particular asthma medication, amphetamines, caffeine, corticosteroids, and drugs used for certain psychiatric and neurological disorders)
• alcohol abuse or withdrawal
• mercury poisoning
• overactive thyroid
• liver or kidney failure
• anxiety or panic.

Symptoms of Physiologic Tremor

Symptoms of tremor may include

• A tremor is involuntary, rhythmic contractions of various muscles. Shaky legs syndrome causes feelings of “vibration”, unsteadiness or imbalance in the legs.
• A rhythmic shaking in the hands, arms, head, legs, or torso
• Balance and muscle coordination problem
• Spasticity and muscle spasm with wasting
• Shaky voice
• Difficulty writing or drawing
• Problems holding and controlling utensils, such as a spoon.
• Begin gradually, usually more prominently on one side of the body
• Worsen with movement
• Usually occur in the hands first, affecting one hand or both hands
• Can include a “yes-yes” or “no-no” motion of the head
• It May be aggravated by emotional stress, fatigue, caffeine, or temperature extremes
• Tremors that get worse during emotional stress
• Tremors that get worse when you move on purpose
• Tremors that lessen with rest
• Balance problems (in rare cases)

What we feel in both legs simultaneously is extreme straining, fatigue, unsteadiness, and a fear of falling. The muscles in our legs become hard, our ankles feel weak and our toes curl under as our legs fail to support us. We can stand for only a short period of time, in some cases only seconds. There is a feeling of panic to find a place to sit, or if possible, walk to gain some relief from our symptoms. Some tremors /shaky legs syndrome may be triggered by or become worse during times of stress or strong emotion, when an individual is physically exhausted, or when a person is in certain postures or makes certain movements.

Diagnosis of Physiologic Tremor

Medical history

During the physical evaluation, a doctor will assess the tremor based on:

• whether the tremor occurs when the muscles are at rest or inaction
• the location of the tremor on the body (and if it occurs on one or both sides of the body)
• the appearance of the tremor (tremor frequency and amplitude).

The doctor will also check other neurological findings such as impaired balance, speech abnormalities, or increased muscle stiffness.  Blood or urine tests can rule out metabolic causes such as thyroid malfunction and certain medications that can cause tremors/shaky legs syndrome.  These tests may also help to identify contributing causes such as drug interactions, chronic alcoholism, or other conditions or diseases.  Diagnostic imaging may help determine if the tremor is the result of damage to the brain.

Treatment of Physiologic Tremor

Non-pharmacological

• Physical, speech-language, and occupational therapy – may help to control tremors and meet daily challenges caused by the tremor.  A physical therapist can help people improve their muscle control, functioning, and strength through coordination, balancing, and other exercises.  Some therapists recommend the use of weights, splints, other adaptive equipment, and special plates and utensils for eating.  Speech-language pathologists can evaluate and treat speech, language, communication, and swallowing disorders.  Occupational therapists can teach individuals new ways of performing activities of daily living that may be affected by tremors.
• Eliminating or reducing tremor-inducing substances such as caffeine and other medication – (such as stimulants) can help improve tremor. Though small amounts of alcohol can improve tremors for some people, tremors can become worse once the effects of the alcohol wear off.
• Interventional Therapy – For patients who fail pharmacologic treatment with the above drugs or are unable to tolerate the side effects, surgical options include deep brain stimulation (DBS), focused ultrasound, or radio-surgical gamma knife thalamotomy to treat persistently disabling limb tremor, and botulinum toxin injections to treat persistently disabling head or vocal cord tremor.
• Deep-brain stimulation – This is the most common surgical treatment for essential tremors. Most series report 70% to 90% hand tremor control. In deep-brain stimulation, electrical stimulation is delivered to the brain through an electrode implanted deep into the ventral intermediate nucleus (VIM) of the thalamus. This is typically done by implanting 4 electrodes in the VIM using stereotactic methods. Computerized programming of the pulse generator is most commonly done with a handheld device after the patient leaves the hospital to optimize the electrode montage, voltage, pulse frequency, and pulse width. Deep-brain stimulation can be done unilaterally or bilaterally depending on the patient’s symptoms. There is an increased risk of speech and balance difficulties with bilateral procedures. If the tremor significantly affects both hands, the dominant hand is targeted, bilateral procedures may be considered.
• Focused ultrasound – Approved by the FDA in 2016, magnetic resonance imaging-guided, high-intensity, focused ultrasound thalamotomy is an innovative method for the treatment of essential tremors. Although it is transcranial and does not require an incision, skull penetration, or an implanted device, it is an invasive therapy that produces a permanent thalamic lesion.
• Radio-surgical gamma knife thalamotomy – Gamma-knife thalamotomy fo­cuses high-energy gamma rays on the ventral intermediate resulting in the death of neurons. It is an unproven treatment that has not generally been adopted due to concerns about potential radiation side effects, including a theoretical, long-term risk of secondary tumor formation.
• Ultrasound Therapy – A new treatment for essential tremors uses magnetic resonance images to deliver focused ultrasound to create a lesion in tiny areas of the brain’s thalamus thought to be responsible for causing the tremors.  The treatment is approved only for those individuals with essential tremors who do not respond well to anticonvulsant or beta-blocking drugs.
• Biofeedback – is a mind-body technique that involves using visual or auditory feedback to teach people to recognize the physical signs and symptoms of stress and anxiety, such as increased heart rate, body temperature, and muscle tension.
• Relaxation techniques – can reduce stress symptoms and help you enjoy a better quality of life, especially if you have an illness. Explore relaxation techniques you can do by yourself.
• Learn to relax – Stress and anxiety tend to make tremors worse, and being relaxed may improve tremors. Although you can’t eliminate all stress from your life, you can change how you react to stressful situations using a range of relaxation techniques, such as massage or meditation.
• Noninvasive techniques – Include gait rehabilitation, visually guided techniques, tendon vibration, weighting extremities, positioning techniques, and manual techniques, all of which can be useful for the recovery of functional activities.[rx]
• Invasive techniques – Thalamic deep brain stimulation can alleviate the tremor in MS, providing better functional performance.[rx] Stereotactic radiosurgery thalamotomy at the nucleus ventralis intermedius with a median maximum dose of 140 Gy also provides good functional outcomes in patients with MS.[rx][rx] Radiofrequency thalamotomy had been successfully used in the past but has been replaced with the newer techniques of radiosurgery and deep brain stimulation as they had fewer adverse effects.
• Physical therapy – can help some patients control their tremors better, as can reducing the intake of substances such as caffeine, which can induce tremors. Many patients experience an increase in the severity of their tremors when they are stressed. Therefore, trying to reduce sources of anxiety and engaging in complementary therapies (such as yoga or aromatherapy) may help some patients.

For example, tremors due to thyroid hyperactivity will improve or even resolve (return to the normal state) with the treatment of thyroid malfunction.  Also, if the tremor is caused by medication, discontinuing the tremor-causing drug may reduce or eliminate this tremor.

Medical Therapy

The therapeutic approach to essential tremors many times follows a trial and error approach, and patients should be challenged by several medications if the first choice is ineffective or associated with debilitating adverse effects. Medical therapy can be divided into first, second, and third-line therapies.

First-line therapy – It is either approved by the FDA or supported by double-blinded, placebo-controlled studies that meet the criteria for the class I evidence. This class of medications includes propranolol and primidone. If both primidone and propranolol are not effective alone, combinations of both may provide relief in selected patients.

Second-line therapy – Second-line therapy is supported by double-blinded, placebo-controlled trials that do not meet other requirements for the class I evidence studies. This includes gabapentin, pregabalin, topiramate, benzodiazepines (clonazepam, alprazolam), beta-blockers (atenolol and metoprolol) and zonisamide.

Third-line therapy – These therapies are based on open-label studies or case series. Drugs in this class include nimodipine and clozapine.

Medication

• Beta-blocking drugs such as propranolol are normally used to treat high blood pressure but they also help treat essential tremors.  Propranolol can also be used in some people with other types of action tremors.  Other beta-blockers that may be used include atenolol, metoprolol, nadolol, and sotalol.
• Anti-seizure medications such as primidone can be effective in people with essential tremors who do not respond to beta-blockers.  Other medications that may be prescribed include gabapentin and topiramate.  However, it is important to note that some anti-seizure medications can cause tremors.
• Tranquilizers (also known as benzodiazepines) such as alprazolam and clonazepam may temporarily help some people with tremors.  However, their use is limited due to unwanted side effects that include sleepiness, poor concentration, and poor coordination.  This can affect the ability of people to perform daily activities such as driving, school, and work.  Also, when taken regularly, tranquilizers can cause physical dependence and when stopped abruptly can cause several withdrawal symptoms.
• Parkinson’s disease medications (levodopa, carbidopa) are used to treat tremors associated with Parkinson’s disease.
• Botulinum toxin – injections can treat almost all types of tremors.  It is especially useful for head tremor, which generally does not respond to medications.  Botulinum toxin is widely used to control dystonic tremors.  Although botulinum toxin injections can improve tremors for roughly three months at a time, they can also cause muscle weakness.  While this treatment is effective and usually well tolerated for head tremors, botulinum toxin treatment in the hands can cause weakness in the fingers.  It can cause a hoarse voice and difficulty swallowing when used to treat voice tremors.

Additional drug therapies that have been used to treat individuals with primary orthostatic tremors include primidone (Mysoline), chlordiazepoxide (Librium), pregabalin (Lyrica), pramipexole (Mirapex), phenobarbital, and valproic acid (Depakote). Drugs commonly used to treat people with Parkinson’s disease (levodopa or pramipexole) may also be prescribed for individuals with primary orthostatic tremors.

Surgery

When people do not respond to drug therapies or have a severe tremor that significantly impacts their daily life, a doctor may recommend surgical interventions such as deep brain stimulation (DBS) or very rarely, thalamotomy.  While DBS is usually well-tolerated, the most common side effects of tremor surgery include dysarthria (trouble speaking) and balance problems.

• Deep brain stimulation (DBS) – is the most common form of surgical treatment of tremors.  This method is preferred because it is effective, has low risk, and treats a broader range of symptoms than thalamotomy.  The treatment uses surgically implanted electrodes to send high-frequency electrical signals to the thalamus, the deep structure of the brain that coordinates and controls some involuntary movements.  A small pulse generating device placed under the skin in the upper chest (similar to a pacemaker) sends electrical stimuli to the brain and temporarily disables the tremor.  DBS is currently used to treat parkinsonian tremors, essential tremors, and dystonia.

• Thalamotomy – is a surgical procedure that involves the precise, permanent destruction of a tiny area in the thalamus.  Currently, surgery is replaced by radiofrequency ablation to treat severe tremors when deep brain surgery is contraindicated—meaning it is unwise as a treatment option or has undesirable side effects.  Radiofrequency ablation uses a radio wave to generate an electric current that heats up a nerve and disrupts its signaling ability for typically six or more months.  It is usually performed on only one side of the brain to improve tremors on the opposite side of the body.  Surgery on both sides is not recommended as it can cause problems with speech.
•  Stereotactic surgical techniques – can be used to create a lesion in the ventral intermediate (VIM) nucleus of the thalamus.

Rehabilitation

Exercise is an important part of healthy living for everyone. For people with tremors, exercise is more than healthy it is a vital component to maintaining balance, mobility, and activities of daily living. Exercise and physical activity can improve many tremors symptoms. These benefits are supported by research.

The tremors show that people with tremors who start exercising earlier and a minimum of 2.5 hours a week, experience a slowed decline in quality of life compared to those who start later. Establishing early exercise habits is essential to overall disease management.

What Type of Exercise Should I Do?

To help manage the symptoms of tremors, be sure your exercise program includes a few key ingredients:

• Aerobic activity
• Strength training
• Balance, agility, and multitasking
• Flexibility

These elements are included in many types of exercise. Biking, running, Tai chi, yoga, Pilates, dance, weight training, non-contact boxing, qi gong, and more — all have positive effects on tremors symptoms.

There is no “exercise prescription” that is right for every person with tremors. The type of exercise you do depends on your symptoms and challenges. For sedentary people, just getting up and moving is beneficial. More active people can build up to the regular, vigorous activity. Many approaches work well to help maintain and improve mobility, flexibility, and balance to ease non-motor tremors symptoms such as depression or constipation.

Researchers in the study did not distinguish between what type of exercise participants did and determined that all types of exercise are beneficial. The most important thing is to do the exercise regularly. We suggest finding an exercise you enjoy and stick with it.

Challenges to Exercising

• People in the early stages of tremors tend to be just as strong and physically fit as healthy individuals of the same age.
• Disease progression can lead to the following physical change:
• Loss of joint flexibility, which can affect balance.
• Decreased muscle strength or deconditioning can affect walking and the ability to stand up from sitting.
• The decline in cardiovascular conditioning, which affects endurance.

Tips for Getting Started

• First, be safe. Before starting an exercise program, consult your neurologist and primary care doctor about concerns and recommendations.
• Ask your doctor or members in your support group to refer to a physical therapist (PT) who knows about tremors. Work together to identify your concerns and limitations. Target exercises to improve them. For most people, a structured exercise program will include aerobic exercise (such as brisk walking) and resistance training (using weights or bands).
• Purchase a pedometer (step-counter) and figure out how many steps you take on average each day, then build up from there. Many smartphones or smartwatches have a built-in pedometer feature or an application that can be downloaded.
• Exercise indoors and outdoors. Change your routine to stay interested and motivated.
• Again, most importantly pick an exercise you enjoy.

What research is being done?

The mission is to seek fundamental knowledge about the brain and nervous system and to use that knowledge to reduce the burden of neurological disease.  The NINDS is a component of the National Institutes of Health (NIH), the leading supporter of biomedical research in the world.

Researchers are working to better understand the underlying brain functions that cause tremors, identify the genetic factors that make individuals more susceptible to the disorder, and develop new and better treatment options.

• Brain functioning – It can be difficult to distinguish between movement disorders such as Parkinson’s disease and essential tremor. These debilitating movement disorders have different prognoses and can respond very differently to available therapies. NINDS researchers are working to identify structural and functional changes in the brain using non-invasive neuroimaging techniques to develop sensitive and specific markers for each of these diseases and then track how they change as each disease progresses. Other researchers are using functional magnetic resonance imaging technology to better understand normal and diseased brain circuit functions and associated motor behaviors.  Scientists hope to design therapies that can restore normal brain circuit function in diseases such as Parkinson’s disease and tremor.
• Genetics – Research has shown that essential tremors may have a strong genetic component affecting multiple generations of families.  NINDS researchers are building on previous genetics work to identify susceptibility genes for familial early-onset (before age 40) essential tremor.  Researchers are focusing on multigenerational, early-onset families to better detect linkages.

Additionally, NINDS scientists are researching the impact of genetic abnormalities on the development of essential tremors.  Previous research that has shown a link between essential tremor and possible genetic variants on chromosome 6 and 11; ongoing research is targeting the impact of other genetic variations in families.

Medications and other treatment methods

While drugs can be effective for some people, approximately 50 percent of individuals do not respond to medication.  In order to develop assistive and rehabilitative tremor-suppressing devices for people with essential tremors, researchers are exploring where and how to minimize or suppress tremors while still allowing for voluntary movements.

Many people with essential tremors respond to ethanol (alcohol); however, it is not clear why or how.  NINDS researchers are studying the impact of ethanol on tremors to determine the correct dosage amount and its physiological impact on the brain and whether other medications without the side effects of ethanol can be effective.

Other NIH researchers hope to identify the source of essential tremors, study the effects of currently available tremor-suppressant drugs on the brain, and develop more targeted and effective therapies.

FAQ

Please answer the following questions to participate in our certified Continuing Medical Education program. Only one answer is possible per question. Please select the answer that is most appropriate.

Question 1

Which of the following constellations of clinical findings is typical of tremor in patients with Parkinson’s disease?

1. bilateral postural tremor

2. unilateral rest tremor and diminished ipsilateral arm swing while walking

3. severe unilateral tremor while holding a cup or glass

4. tremor that only appears when the patient writes

5. postural tremor of both hands and ataxic gait

Question 2

A 25-year-old man has a mild postural tremor of both hands that improves when he drinks alcohol. His mother had the same condition. What can you advise him?

1. He should definitely be evaluated for possible early Parkinson’s disease.

2. He must get treatment now, as otherwise the condition could worsen.

3. If treatment is indicated, propranolol or primidone could be given.

4. Relaxation exercises and physiotherapy are effective treatment options.

5. Genetic testing is needed to confirm the diagnosis of essential tremor.

Question 3

A man who received the diagnosis of multiple sclerosis two years ago presents to you with the new onset of tremor. What constellation of clinical findings is typical of tremor due to multiple sclerosis?

1. rest tremor, only occasionally observable when the patient is excited

2. a tremor that appears sometimes on the left side, sometimes on the right

3. a tremor that is only present in the morning

4. a swaying, broad-based gait and an intention tremor

5. a postural tremor that is easily suppressed by voluntary effort

Question 4

A 55-year-old man with essential tremor says that he can no longer feed himself because of tremor, can dress himself only with great difficulty, and has not had legible handwriting for many years. Drug treatment as recommended in the relevant clinical guidelines brings only slight improvement. What can you advise the patient about the option of surgical treatment?

1. Deep brain stimulation (DBS) might help but is not available in Germany.

2. DBS is an experimental technique that is only performed in clinical trials.

3. DBS is indicated only to treat Parkinson’s disease and plays no role in the treatment of essential tremor.

4. DBS has a high chance of success in this situation; it is now established as a standard treatment for essential tremor.

5. DBS is no more effective than pharmacotherapy for this indication.

Question 5

What information is most important for the diagnostic classification of a tremor syndrome?

1. the clinical findings

2. brain magnetic resonance imaging (MRI) with fine cerebellar sections

3. nuclear-medical visualization of brain perfusion

4. ultrasonography of the basal ganglia

5. measurement of serum drug levels

Question 6

What findings indicate that tremor may be psychogenic?

1. no evidence of essential tremor or Parkinson’s disease on brain MRI

2. a longstanding marital conflict

3. a tremor of inconstant location that diminishes on distraction and is found to be irregular on tremor analysis

4. a clearly identifiable underlying psychological conflict

5. remission after psychotherapy

Question 7

When can tremor be treated surgically?

1. When the patient is unwilling to take drugs to treat tremor.

2. When the patient is under 50 years old.

3. When the tremor cannot be adequately suppressed by drugs and there is no contraindication to surgery.

4. When the patient is willing to see a neurosurgeon once a week so that brain stimulation can be performed.

5. When the patient is willing to assume the cost of weekly battery changes.

Question 8

What must be borne in mind with respect to drug treatment for various tremor syndromes?

1. That the treatment is based on the clinical findings and not on the underlying disease causing tremor.

2. That causally directed treatment is generally possible only for drug-induced tremors or tremors due to metabolic disturbance.

3. That parkinsonian tremor responds best to anticholinergic drugs and does not respond at all to the classic dopamine preparations.

4. That the cerebellar tremor of multiple sclerosis is treated in exactly the same way as essential tremor.

5. That essential tremor is usually medically intractable.

Question 9

What drugs can induce tremor?

1. lithium, valproic acid, cyclosporine A

2. carbamazepine, propranolol, Seroxat

3. aspirin, diclofenac, paracetamol

4. penicillin, erythromycin, cephalosporin

5. antilipid drugs, antidiabetic drugs

Question 10

What is the drug, or drug class, of first choice for the treatment of parkinsonian tremor?

1. dopaminergic drugs

2. propanolol

3. primidone

4. gabapentin

5. ondansetron

References

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Trigeminal Neuralgia Type 2 (TN2)/Trigeminal neuralgia (TN), also called tic douloureux, is a chronic pain condition that affects the trigeminal or 5th cranial nerve, one of the most widely distributed nerves in the head. TN is a form of neuropathic pain (pain associated with nerve injury or nerve lesion.) The typical or “classic” form of the disorder (called “Type 1” or TN1) causes extreme, sporadic, sudden burning, or shock-like facial pain that lasts anywhere from a few seconds to as long as two minutes per episode.  These attacks can occur in quick succession, in volleys lasting as long as two hours.  The “atypical” form of the disorder (called “Type 2” or TN2), is characterized by constant aching, burning, stabbing pain of somewhat lower intensity than Type 1.  Both forms of pain may occur in the same person, sometimes at the same time. The intensity of pain can be physically and mentally incapacitating.

TRIGEMINAL NEURALGIA is an extremely severe unilateral episodic facial pain that tends to come and go unpredictably in sudden shock-like attacks. The pain is normally triggered, for example by light touch, and is described as stabbing, shooting, excruciating or burning. It usually lasts for a few seconds but there can be many bursts of pain in quick succession.  There can be slight variations of trigeminal neuralgia which require different treatments.

Types of Trigeminal Neuralgia

The typical or “classic” form of the disorder (called TN1) causes extreme, sporadic, sudden burning or shock-like facial pain in the areas of the face where the branches of the nerve are distributed – lips, eyes, nose, scalp, forehead, upper jaw, and lower jaw. Trigeminal neuralgia can progress and cause longer, more frequent bouts of searing pain. You may feel as though your pain came out of nowhere. Some people initially think their pain is a toothache or migraine headache.

The “atypical” form of the disorder (called TN2), is characterized by constant aching, burning, stabbing pain of somewhat lower intensity than TN1. Both forms of pain may occur in the same person, sometimes at the same time

The subtypes of TN are defined by ICHD-3 as follows[rx]

• Classic TN: This is secondary to neuromuscular compression and fulfilling the criteria above. This requires demonstration of the compression on an MRI or during the surgery for neuromuscular compression, with associated morphological changes in the trigeminal nerve root.
• Secondary TN: This is defined as TN secondary to an underlying disease. Some of the reported causes are multiple sclerosis, arteriovenous malformation, and cerebellopontine angle tumor.
• Idiopathic TN: This is defined as TN with no abnormalities seen on MRI or electrophysiological tests.

The trigeminal nerve is one of 12 pairs of nerves that are attached to the brain. The nerve has three branches that conduct sensations from the upper, middle, and lower portions of the face, as well as the oral cavity, to the brain. The ophthalmic, or upper, branch supplies sensation to most of the scalp, forehead, and front of the head. The maxillary, or middle, branch stimulates the cheek, upper jaw, top lip, teeth, and gums, and to the side of the nose. The mandibular, or lower, branch supplies nerves to the lower jaw, teeth and gums, and bottom lip. More than one nerve branch can be affected by the disorder. Rarely, both sides of the face may be affected at different times in an individual, or even more rarely at the same time (called bilateral TN).

What causes trigeminal neuralgia?

TN is associated with a variety of conditions. TN can be caused by a blood vessel pressing on the trigeminal nerve as it exits the brain stem. This compression causes the wearing away or damage to the protective coating around the nerve (the myelin sheath). TN symptoms can also occur in people with multiple sclerosis, a disease that causes deterioration of the trigeminal nerve’s myelin sheath. Rarely, symptoms of TN may be caused by nerve compression from a tumor, or a tangle of arteries and veins called an arteriovenous malformation. Injury to the trigeminal nerve (perhaps the result of sinus surgery, oral surgery, stroke, or facial trauma) may also produce neuropathic facial pain.

The exact cause of trigeminal neuralgia remains unknown.[rx] The majority of cases are referred to as idiopathic, although many are associated with vascular compression of the trigeminal nerve close to its exit from the brainstem by an aberrant loop of an artery or vein. A minority of cases are due to conditions like multiple sclerosis or nerve compression by a tumor.[rx] Some rare causes of trigeminal neuralgia include focal arachnoid thickening, adhesion, traction, tethering or torsion, fibrous ring around the root, cerebellopontine angle tumors, brain stem infarction, aneurysm, and arteriovenous malformation.[rx][rx][rx][rx]

The trigeminal nerve starts at the pons. Most cases of trigeminal neuralgia are due to the compression of the trigeminal nerve root, within a few millimeters of its entry into the pons. Between 80% and 90% of the cases of TN are caused by compression by an adjacent artery or a vein.[rx] The blood vessel, which has been mostly implicated in about 75% to 80% of the cases, is the superior cerebellar artery. Other blood vessels that are known to cause TN include the anterior inferior cerebellar artery, the vertebral artery, and the petrosal vein.

Vascular Theory

Generally, it has been assumed that vascular contact at the root entry zone causes trigeminal neuralgia; however, TN also may be caused by contact at a transition zone between the central and peripheral myelin. Reportedly, the most common artery involved in this condition is the superior cerebellar artery, as seen in 75% to 80% of TN cases. Persistent primitive trigeminal artery variant, an anomaly that occurs between the carotid and basilar arteries or aneurysms of the persistent primitive trigeminal artery, vertebrobasilar dolichoectasia can cause TN. Sharper trigeminal-pontine angle cisterns and smaller cerebellopontine angle cisterns may facilitate neurovascular compression (NVC).[rx][rx][rx][rx][rx]

Extracranial Causes

The most common extracranial cause of trigeminal neuralgia is a perineural spread of head and neck malignancies, commonly squamous cell carcinoma, adenoid cystic carcinoma, lymphoma, melanoma, and sarcoma.[rx][rx][rx][rx][rx]

What are the symptoms of trigeminal neuralgia?

Pain varies, depending on the type of TN, and may range from sudden, severe, and stabbing to a more constant, aching, burning sensation. The intense flashes of pain can be triggered by vibration or contact with the cheek (such as when shaving, washing the face, or applying makeup), brushing teeth, eating, drinking, talking, or being exposed to the wind. The pain may affect a small area of the face or may spread. Bouts of pain rarely occur at night, when the affected individual is sleeping.

TN is typified by attacks that stop for a period of time and then return, but the condition can be progressive. The attacks often worsen over time, with fewer and shorter pain-free periods before they recur. Eventually, the pain-free intervals disappear and medication to control the pain becomes less effective. The disorder is not fatal but can be debilitating. Due to the intensity of the pain, some individuals may avoid daily activities or social contacts because they fear an impending attack.

The trigeminal nerve is the fifth cranial nerve. It is responsible for the sensory supply of the face and the motor and sensory supply to the muscles of mastication. The trigeminal nerve starts at the pons and divides into three branches[rx]:

• Ophthalmic (V1): Supplies the eye, upper eyelid, and the forehead
• Maxillary (V2): Supplies lower eyelid, cheek, nostril, upper lip, and upper gum
• Mandibular (V3): Supplies the lower lip, lower gum, jaw and muscles of mastication

Trigeminal neuralgia symptoms may include one or more of these patterns:

• Episodes of severe, shooting or jabbing pain that may feel like an electric shock
• Spontaneous attacks of pain or attacks triggered by things such as touching the face, chewing, speaking or brushing teeth
• Bouts of pain lasting from a few seconds to several minutes
• Episodes of several attacks lasting days, weeks, months, or longer — some people have periods when they experience no pain
• Constant aching, burning feeling that may occur before it evolves into the spasm-like pain of trigeminal neuralgia
• Pain in areas supplied by the trigeminal nerve, including the cheek, jaw, teeth, gums, lips, or less often the eye and forehead
• Pain affecting one side of the face at a time though may rarely affect both sides of the face
• Pain focused in one spot or spread in a wider pattern
• Attacks that become more frequent and intense over time
• The attacks happen several times a day or a week, followed by periods during which you have none at all. These pain-free periods are known as remission.
• The pain usually affects only one side of the face.
• The attacks happen more often over time, and the pain can worsen.
• You feel the pain mostly in your cheek, jaw, teeth, gums, and lips. The eyes and forehead are affected less often.

Symptom triggers

Attacks of trigeminal neuralgia can be triggered by certain actions or movements, such as:

• talking
• smiling
• chewing
• brushing your teeth
• washing your face
• a light touch
• shaving or putting on make-up
• swallowing
• kissing
• a cool breeze or air conditioning
• head movements
• vibrations, such as walking or traveling in a car

However, pain can happen spontaneously with no trigger whatsoever.

The symptoms of several pain disorders are similar to those of trigeminal neuralgia. The most common mimicker of TN is trigeminal neuropathic pain (TNP). TNP results from an injury or damage to the trigeminal nerve. TNP pain is generally described as being constant, dull and burning. Attacks of sharp pain can also occur, commonly triggered by touch. Additional mimickers include:

• Temporal tendinitis
• Ernest syndrome (injury of the stylomandibular ligament
• Occipital neuralgia
• Cluster headaches/ migraines
• Giant cell arteritis
• Dental pain
• Post-herpetic neuralgia
• Glossopharyngeal neuralgia
• Sinus infection
• Ear infection
• Temporomandibular joint syndrome (TMJ)

Diagnosis of Child Trigeminal Neuralgia

TN diagnosis is based primarily on the person’s history and description of symptoms, along with results from physical and neurological examinations. Other disorders that cause facial pain should be ruled out before TN is diagnosed. Some disorders that cause facial pain include post-herpetic neuralgia (nerve pain following an outbreak of shingles), cluster headaches, and temporomandibular joint disorder (TMJ, which causes pain and dysfunction in the jaw joint and muscles that control jaw movement).  Because of overlapping symptoms and the large number of conditions that can cause facial pain, obtaining a correct diagnosis is difficult, but finding the cause of the pain is important as the treatments for different types of pain may differ.

Most people with TN eventually will undergo a magnetic resonance imaging (MRI) scan to rule out a tumor or multiple sclerosis as the cause of their pain. This scan may or may not clearly show a blood vessel compressing the nerve. Special MRI imaging procedures can reveal the presence and severity of compression of the nerve by a blood vessel.

A diagnosis of classic trigeminal neuralgia may be supported by an individual’s positive response to a short course of antiseizure medication. Diagnosis of TN2 is more complex and difficult but tends to be supported by a positive response to low doses of tricyclic antidepressant medications (such as amitriptyline and nortriptyline), similar to other neuropathic pain diagnoses.

Your doctor may conduct many tests to diagnose trigeminal neuralgia and determine underlying causes for your condition, including:

• A neurological examination. Touching and examining parts of your face can help your doctor determine exactly where the pain is occurring and — if you appear to have trigeminal neuralgia — which branches of the trigeminal nerve may be affected. Reflex tests also can help your doctor determine if your symptoms are caused by a compressed nerve or another condition.
• Magnetic resonance imaging (MRI). Your doctor may order an MRI scan of your head to determine if multiple sclerosis or a tumor is causing trigeminal neuralgia. In some cases, your doctor may inject a dye into a blood vessel to view the arteries and veins and highlight blood flow (magnetic resonance angiogram).

One published a set of guidelines for diagnosing TN is from the International Headache Society.

For classical TN

• Paroxysmal attacks of pain lasting from a fraction of a second to two minutes, affecting one or more divisions of the trigeminal nerve, and fulfilling criteria 2 and 3.
• Pain has at least one of the following characteristics:
  • Intense, sharp, superficial or stabbing
  • Precipitated from trigger zones or by trigger factors
• Attacks are stereotyped in the individuals patient
• There is no clinically evident neurologic deficit
• Not attributed to another disorder

For symptomatic TN

• Paroxysmal attacks of pain lasting from a fraction of a second to two minutes, with or without persistence of aching between paroxysms, affecting one or more divisions of the trigeminal nerve, and fulfilling criteria 2 and 3.
• Pain has at least one of the following characteristics
  • Intense, sharp, superficial or stabbing
  • Precipitated from trigger zones or by trigger factors.
• Attacks are stereotyped in the individual patient
• A causative lesion, other than vascular compression, has been demonstrated by special investigations and/or posterior fossa exploration.

Treatment of Child Trigeminal Neuralgia

Treatment options include medicines, surgery, and complementary approaches.

Pharmacologic Therapy

• The first-line treatment – for patients with classic TN and idiopathic TN is pharmacologic therapy. The most commonly used medication is the anticonvulsant drug, carbamazepine.[rx] It is usually started at a low dose, and the dose is gradually increased until it controls the pain. It controls pain for most people in the early stages of the disease. However, in some patients, the effectiveness of carbamazepine decreases over time. Possible side effects of carbamazepine include drowsiness, dizziness, double vision, and nausea. In patients with Asian ancestry, before starting carbamazepine, testing for the HLA-B allele is recommended, as its presence increases the risk of development of toxic epidermal necrolysis or Stevens-Johnson syndrome.[rx]

• Oxcarbazepine – is a newer drug and is being increasingly used as first-line therapy for TN in patients who do not respond to or who cannot tolerate carbamazepine (200 to 1200 mg/day) and oxcarbazepine (600 to 1800 mg/day). Possible side effects include double vision and dizziness. It can also cause hyponatremia. It should also be avoided in patients with the HLA-B 15:02 allele.

• Muscle relaxant – Baclofen is a muscle relaxant that can be used to treat TN. Baclofen, lamotrigine, clonazepam, topiramate, phenytoin, gabapentin, pregabalin, and sodium valproate can be used. Side effects include dizziness, sedation, and dyspepsia.

• Seizures, anticonvulsant medication – used to treat epilepsy and to delay or prevent the recurrence of depressive episodes in bipolar disorder. For epilepsy, this includes focal seizures, tonic-clonic seizures, and seizures in Lennox-Gastaut syndrome and medication lamotrigine, phenytoin, gabapentin, clonazepam, and valproic acid. Lamotrigine (200 to 400 mg/day), pregabalin (150 to 600 mg/day), gabapentin (1800 to 4200 mg/day), or topiramate (100 to 400 mg/day) may be considered. If the combination therapy fails, a switch to baclofen (40 to 80 mg/day) may be considered.[rx]

• Eslicarbazepine – an active metabolite of oxcarbazepine, and the new Nav1.7 blocker, vixotrigine, are being explored for pain relief in TN.[rx]

• Anticonvulsant medicines—used to block nerve firing—are generally effective in treating TN1 but often less effective in TN2. These drugs include carbamazepine, oxcarbazepine, topiramate, gabapentin, pregabalin, clonazepam, phenytoin, lamotrigine, and valproic acid.

• Tricyclic antidepressants – such as amitriptyline or nortriptyline can be used to treat pain. Common analgesics and opioids are not usually helpful in treating the sharp, recurring pain caused by TN1, although some individuals with TN2 do respond to opioids.  Eventually, if medication fails to relieve pain or produces intolerable side effects such as cognitive disturbances, memory loss, excess fatigue, bone marrow suppression, or allergy, then surgical treatment may be indicated. Since TN is a progressive disorder that often becomes resistant to medication over time, individuals often seek surgical treatment.

• Botulinum Toxin Injections – This can be beneficial for some patients, particularly the middle-aged and the elderly, who are refractory to medical therapy or who cannot tolerate medical therapy due to their side effects.[rx]

• Tetracaine nerve block – may be used as an additional treatment after carbamazepine, as can acupuncture and/or peripheral nerve stimulation.[rx] Patients with secondary TN also can respond well to pharmacotherapy. However, it is recommended to treat the underlying lesion or disease.

• There is controversy around opiate use such as morphine and oxycodone for treatment of TN, with varying evidence on its effectiveness for neuropathic pain. Generally, opioids are considered ineffective against TN and thus should not be prescribed.^[rx]

Surgical Therapy

• Microvascular decompression – This is one of the most common procedures used to treat trigeminal neuralgia. This is beneficial for patients with TN, where compression of the nerve root is the cause. [rx]This involves craniotomy and posterior fossa exploration for identifying and moving the blood vessel that is compressing the trigeminal nerve. A soft cushion is then inserted between the nerve and the vessel, to allow the nerve to recover, which eventually relieves the pain. In some patients, this procedure can result in sustained pain relief for greater than 10 years. Though this is the most effective procedure, it is also the most invasive one. Some of the complications associated with it are decreased hearing, cerebellar hematoma, CSF leaks, infarction, and facial weakness. It is believed to be the most effective long-term surgical treatment available currently for patients with TN.[rx]
• Ablative procedures include rhizotomy–  with thermocoagulation, chemical injection, or mechanical balloon compression. These procedures involve damaging the trigeminal nerve root, thereby interrupting the pain transmission signals to the brain. Rhizotomy with thermocoagulation uses an electrode to apply heat to damage the nerve fibers. Chemical rhizotomy involves injecting the chemical, glycerol to the trigeminal nerve, thereby damaging it. Balloon compression involves inserting a tiny balloon to the point of location of nerve fibers.[rx][rx] This balloon, on inflation, damages the nerve fibers. Some of the associated complications are postoperative dysesthesia, corneal numbness, sensory loss in trigeminal nerve distribution, and anesthesia Dolorosa.
• Radiosurgery – This procedure involves using radiosurgery instrumentation.[rx][rx][rx] This is a non-invasive procedure, wherein, a highly concentrated dose of ionizing radiation is delivered to a precise target at the trigeminal nerve root. The radiation creates a lesion near the nerve root, thereby interrupting the pain signals from transmission to the brain. The formation of the lesion can be slow, and hence the pain relief using this procedure is delayed by up to several weeks or months. As this is one of the least invasive procedures, it can be repeated in patients who have a recurrence of pain. Some of the associated complications can be facial sensory loss and paresthesias.
• Peripheral neurectomy and nerve block – The neurectomy can be performed on peripheral branches of the trigeminal nerve like the supraorbital, infraorbital, lingual, and alveolar nerves.[rx] This can be accomplished by alcohol injection, incision, cryotherapy, or radiofrequency lesioning. Peripheral neurectomy can be safe in elderly patients in remote and rural areas, where neurosurgical facilities are not readily available.[rx] However, the evidence regarding these peripheral techniques for trigeminal neuralgia is inconclusive.
• A rhizotomy (rhizolysis)–  is a procedure in which nerve fibers are damaged to block pain. A rhizotomy for TN always causes some degree of sensory loss and facial numbness. Several forms of rhizotomy are available to treat trigeminal neuralgia:
• Balloon compression – works by injuring the insulation on nerves that are involved with the sensation of light touch on the face. The procedure is performed in an operating room under general anesthesia. A tube called a cannula is inserted through the cheek and guided to where one branch of the trigeminal nerve passes through the base of the skull. A soft catheter with a balloon tip is threaded through the cannula and the balloon is inflated to squeeze part of the nerve against the hard edge of the brain covering (the dura) and the skull. After about a minute the balloon is deflated and removed, along with the catheter and cannula. Balloon compression is generally an outpatient procedure, although sometimes the patient may be kept in the hospital overnight. Pain relief usually lasts one to two years.
• Glycerol injection – is also generally an outpatient procedure in which the individual is sedated with intravenous medication. A thin needle is passed through the cheek, next to the mouth, and guided through the opening in the base of the skull where the third division of the trigeminal nerve (mandibular) exits. The needle is moved into the pocket of spinal fluid (cistern) that surrounds the trigeminal nerve center (or ganglion, the central part of the nerve from which the nerve impulses are transmitted to the brain). The procedure is performed with the person sitting up, since glycerol is heavier than spinal fluid and will then remain in the spinal fluid around the ganglion. The glycerol injection bathes the ganglion and damages the insulation of trigeminal nerve fibers. This form of rhizotomy is likely to result in the recurrence of pain within a year to two years. However, the procedure can be repeated multiple times.
• Radiofrequency thermal lesioning – (also known as “RF Ablation” or “RF Lesion”) is most often performed on an outpatient basis. The individual is anesthetized and a hollow needle is passed through the cheek through the same opening at the base of the skull where the balloon compression and glycerol injections are performed. The individual is briefly awakened and a small electrical current is passed through the needle, causing tingling in the area of the nerve where the needle tips rest. When the needle is positioned so that the tingling occurs in the area of TN pain, the person is then sedated and the nerve area is gradually heated with an electrode, injuring the nerve fibers.  The electrode and needle are then removed and the person is awakened. The procedure can be repeated until the desired amount of sensory loss is obtained; usually a blunting of sharp sensation, with preservation of touch. Approximately half of the people have symptoms that reoccur three to four years following RF lesioning. Production of more numbness can extend the pain relief even longer, but the risks of anesthesia dolorosa also increase.
• Stereotactic radiosurgery – (Gamma Knife, CyberKnife) uses computer imaging to direct highly focused beams of radiation at the site where the trigeminal nerve exits the brain stem. This causes the slow formation of a lesion on the nerve that disrupts the transmission of sensory signals to the brain. People usually leave the hospital the same day or the next day following treatment but won’t typically experience relief from pain for several weeks (or sometimes several months) following the procedure.  The International Radiosurgery Association reports that between 50 and 78 percent of people with TN who are treated with Gamma Knife radiosurgery experience “excellent” pain relief within a few weeks following the procedure. For individuals who were treated successfully, almost half have a recurrence of pain within three years.
• Decompression Surgery –  is the most invasive of all surgeries for TN, but also offers the lowest probability that pain will return. About half of individuals undergoing MVD for TN will experience recurrent pain within 12 to 15 years.  This inpatient procedure, which is performed under general anesthesia, requires that a small opening be made through the mastoid bone behind the ear. While viewing the trigeminal nerve through a microscope or endoscope, the surgeon moves away from the vessel (usually an artery) that is compressing the nerve and places a soft cushion between the nerve and the vessel. Unlike rhizotomies, the goal is not to produce numbness in the face after this surgery. Individuals generally recuperate for several days in the hospital following the procedure, and will generally need to recover for several weeks after the procedure.
• A neurectomy (also called partial nerve section) – which involves cutting part of the nerve, may be performed near the entrance point of the nerve at the brain stem during an attempted microvascular decompression if no vessel is found to be pressing on the trigeminal nerve. Neurectomies also may be performed by cutting superficial branches of the trigeminal nerve in the face. When done during microvascular decompression, a neurectomy will cause more long-lasting numbness in the area of the face that is supplied by the nerve or nerve branch that is cut. However, when the operation is performed in the face, the nerve may grow back and in time sensation may return.  With neurectomy, there is risk of creating anesthesia Dolorosa.

Surgical treatment for TN2 is usually more problematic than for TN1, particularly where vascular compression is not detected in brain imaging prior to a proposed procedure. Many neurosurgeons advise against the use of MVD or rhizotomy in individuals for whom TN2 symptoms predominate over TN1, unless vascular compression has been confirmed. MVD for TN2 is also less successful than for TN1.

Some individuals manage trigeminal neuralgia using complementary techniques, usually in combination with drug treatment. These therapies offer varying degrees of success. Some people find that low-impact exercise, yoga, creative visualization, aromatherapy, or meditation may be useful in promoting well-being. Other options include acupuncture, upper cervical chiropractic, biofeedback, vitamin therapy, and nutritional therapy. Some people report modest pain relief after injections of botulinum toxin to block the activity of sensory nerves.

Chronic pain from TN is frequently very isolating and depressing for the individual. Conversely, depression and sleep disturbance may render individuals more vulnerable to pain and suffering. Some individuals benefit from supportive counseling or therapy by a psychiatrist or psychologist. However, there is no evidence that TN is psychogenic in origin or caused by depression, and persons with TN require effective medical or surgical treatment for their pain.

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Trigeminal Neuralgia Type 1 (TN1)/Trigeminal neuralgia (TN), also called tic douloureux, is a chronic pain condition that affects the trigeminal or 5th cranial nerve, one of the most widely distributed nerves in the head. TN is a form of neuropathic pain (pain associated with nerve injury or nerve lesion.) The typical or “classic” form of the disorder (called “Type 1” or TN1) causes extreme, sporadic, sudden burning, or shock-like facial pain that lasts anywhere from a few seconds to as long as two minutes per episode.  These attacks can occur in quick succession, in volleys lasting as long as two hours.  The “atypical” form of the disorder (called “Type 2” or TN2), is characterized by constant aching, burning, stabbing pain of somewhat lower intensity than Type 1.  Both forms of pain may occur in the same person, sometimes at the same time. The intensity of pain can be physically and mentally incapacitating.

TRIGEMINAL NEURALGIA is an extremely severe unilateral episodic facial pain that tends to come and go unpredictably in sudden shock-like attacks. The pain is normally triggered, for example by light touch, and is described as stabbing, shooting, excruciating or burning. It usually lasts for a few seconds but there can be many bursts of pain in quick succession.  There can be slight variations of trigeminal neuralgia which require different treatments.

Types of Trigeminal Neuralgia

The typical or “classic” form of the disorder (called TN1) causes extreme, sporadic, sudden burning or shock-like facial pain in the areas of the face where the branches of the nerve are distributed – lips, eyes, nose, scalp, forehead, upper jaw, and lower jaw. Trigeminal neuralgia can progress and cause longer, more frequent bouts of searing pain. You may feel as though your pain came out of nowhere. Some people initially think their pain is a toothache or migraine headache.

The “atypical” form of the disorder (called TN2), is characterized by constant aching, burning, stabbing pain of somewhat lower intensity than TN1. Both forms of pain may occur in the same person, sometimes at the same time

The subtypes of TN are defined by ICHD-3 as follows[rx]

• Classic TN: This is secondary to neuromuscular compression and fulfilling the criteria above. This requires demonstration of the compression on an MRI or during the surgery for neuromuscular compression, with associated morphological changes in the trigeminal nerve root.
• Secondary TN: This is defined as TN secondary to an underlying disease. Some of the reported causes are multiple sclerosis, arteriovenous malformation, and cerebellopontine angle tumor.
• Idiopathic TN: This is defined as TN with no abnormalities seen on MRI or electrophysiological tests.

The trigeminal nerve is one of 12 pairs of nerves that are attached to the brain. The nerve has three branches that conduct sensations from the upper, middle, and lower portions of the face, as well as the oral cavity, to the brain. The ophthalmic, or upper, branch supplies sensation to most of the scalp, forehead, and front of the head. The maxillary, or middle, branch stimulates the cheek, upper jaw, top lip, teeth, and gums, and to the side of the nose. The mandibular, or lower, branch supplies nerves to the lower jaw, teeth and gums, and bottom lip. More than one nerve branch can be affected by the disorder. Rarely, both sides of the face may be affected at different times in an individual, or even more rarely at the same time (called bilateral TN).

What causes trigeminal neuralgia?

TN is associated with a variety of conditions. TN can be caused by a blood vessel pressing on the trigeminal nerve as it exits the brain stem. This compression causes the wearing away or damage to the protective coating around the nerve (the myelin sheath). TN symptoms can also occur in people with multiple sclerosis, a disease that causes deterioration of the trigeminal nerve’s myelin sheath. Rarely, symptoms of TN may be caused by nerve compression from a tumor, or a tangle of arteries and veins called an arteriovenous malformation. Injury to the trigeminal nerve (perhaps the result of sinus surgery, oral surgery, stroke, or facial trauma) may also produce neuropathic facial pain.

The exact cause of trigeminal neuralgia remains unknown.[rx] The majority of cases are referred to as idiopathic, although many are associated with vascular compression of the trigeminal nerve close to its exit from the brainstem by an aberrant loop of an artery or vein. A minority of cases are due to conditions like multiple sclerosis or nerve compression by a tumor.[rx] Some rare causes of trigeminal neuralgia include focal arachnoid thickening, adhesion, traction, tethering or torsion, fibrous ring around the root, cerebellopontine angle tumors, brain stem infarction, aneurysm, and arteriovenous malformation.[rx][rx][rx][rx]

The trigeminal nerve starts at the pons. Most cases of trigeminal neuralgia are due to the compression of the trigeminal nerve root, within a few millimeters of its entry into the pons. Between 80% and 90% of the cases of TN are caused by compression by an adjacent artery or a vein.[rx] The blood vessel, which has been mostly implicated in about 75% to 80% of the cases, is the superior cerebellar artery. Other blood vessels that are known to cause TN include the anterior inferior cerebellar artery, the vertebral artery, and the petrosal vein.

Vascular Theory

Generally, it has been assumed that vascular contact at the root entry zone causes trigeminal neuralgia; however, TN also may be caused by contact at a transition zone between the central and peripheral myelin. Reportedly, the most common artery involved in this condition is the superior cerebellar artery, as seen in 75% to 80% of TN cases. Persistent primitive trigeminal artery variant, an anomaly that occurs between the carotid and basilar arteries or aneurysms of the persistent primitive trigeminal artery, vertebrobasilar dolichoectasia can cause TN. Sharper trigeminal-pontine angle cisterns and smaller cerebellopontine angle cisterns may facilitate neurovascular compression (NVC).[rx][rx][rx][rx][rx]

Extracranial Causes

The most common extracranial cause of trigeminal neuralgia is a perineural spread of head and neck malignancies, commonly squamous cell carcinoma, adenoid cystic carcinoma, lymphoma, melanoma, and sarcoma.[rx][rx][rx][rx][rx]

What are the symptoms of trigeminal neuralgia?

Pain varies, depending on the type of TN, and may range from sudden, severe, and stabbing to a more constant, aching, burning sensation. The intense flashes of pain can be triggered by vibration or contact with the cheek (such as when shaving, washing the face, or applying makeup), brushing teeth, eating, drinking, talking, or being exposed to the wind. The pain may affect a small area of the face or may spread. Bouts of pain rarely occur at night, when the affected individual is sleeping.

TN is typified by attacks that stop for a period of time and then return, but the condition can be progressive. The attacks often worsen over time, with fewer and shorter pain-free periods before they recur. Eventually, the pain-free intervals disappear and medication to control the pain becomes less effective. The disorder is not fatal but can be debilitating. Due to the intensity of the pain, some individuals may avoid daily activities or social contacts because they fear an impending attack.

The trigeminal nerve is the fifth cranial nerve. It is responsible for the sensory supply of the face and the motor and sensory supply to the muscles of mastication. The trigeminal nerve starts at the pons and divides into three branches[rx]:

• Ophthalmic (V1): Supplies the eye, upper eyelid, and the forehead
• Maxillary (V2): Supplies lower eyelid, cheek, nostril, upper lip, and upper gum
• Mandibular (V3): Supplies the lower lip, lower gum, jaw and muscles of mastication

Trigeminal neuralgia symptoms may include one or more of these patterns:

• Episodes of severe, shooting or jabbing pain that may feel like an electric shock
• Spontaneous attacks of pain or attacks triggered by things such as touching the face, chewing, speaking or brushing teeth
• Bouts of pain lasting from a few seconds to several minutes
• Episodes of several attacks lasting days, weeks, months, or longer — some people have periods when they experience no pain
• Constant aching, burning feeling that may occur before it evolves into the spasm-like pain of trigeminal neuralgia
• Pain in areas supplied by the trigeminal nerve, including the cheek, jaw, teeth, gums, lips, or less often the eye and forehead
• Pain affecting one side of the face at a time though may rarely affect both sides of the face
• Pain focused in one spot or spread in a wider pattern
• Attacks that become more frequent and intense over time
• The attacks happen several times a day or a week, followed by periods during which you have none at all. These pain-free periods are known as remission.
• The pain usually affects only one side of the face.
• The attacks happen more often over time, and the pain can worsen.
• You feel the pain mostly in your cheek, jaw, teeth, gums, and lips. The eyes and forehead are affected less often.

Symptom triggers

Attacks of trigeminal neuralgia can be triggered by certain actions or movements, such as:

• talking
• smiling
• chewing
• brushing your teeth
• washing your face
• a light touch
• shaving or putting on make-up
• swallowing
• kissing
• a cool breeze or air conditioning
• head movements
• vibrations, such as walking or traveling in a car

However, pain can happen spontaneously with no trigger whatsoever.

The symptoms of several pain disorders are similar to those of trigeminal neuralgia. The most common mimicker of TN is trigeminal neuropathic pain (TNP). TNP results from an injury or damage to the trigeminal nerve. TNP pain is generally described as being constant, dull and burning. Attacks of sharp pain can also occur, commonly triggered by touch. Additional mimickers include:

• Temporal tendinitis
• Ernest syndrome (injury of the stylomandibular ligament
• Occipital neuralgia
• Cluster headaches/ migraines
• Giant cell arteritis
• Dental pain
• Post-herpetic neuralgia
• Glossopharyngeal neuralgia
• Sinus infection
• Ear infection
• Temporomandibular joint syndrome (TMJ)

Diagnosis of Child Trigeminal Neuralgia

TN diagnosis is based primarily on the person’s history and description of symptoms, along with results from physical and neurological examinations. Other disorders that cause facial pain should be ruled out before TN is diagnosed. Some disorders that cause facial pain include post-herpetic neuralgia (nerve pain following an outbreak of shingles), cluster headaches, and temporomandibular joint disorder (TMJ, which causes pain and dysfunction in the jaw joint and muscles that control jaw movement).  Because of overlapping symptoms and the large number of conditions that can cause facial pain, obtaining a correct diagnosis is difficult, but finding the cause of the pain is important as the treatments for different types of pain may differ.

Most people with TN eventually will undergo a magnetic resonance imaging (MRI) scan to rule out a tumor or multiple sclerosis as the cause of their pain. This scan may or may not clearly show a blood vessel compressing the nerve. Special MRI imaging procedures can reveal the presence and severity of compression of the nerve by a blood vessel.

A diagnosis of classic trigeminal neuralgia may be supported by an individual’s positive response to a short course of antiseizure medication. Diagnosis of TN2 is more complex and difficult but tends to be supported by a positive response to low doses of tricyclic antidepressant medications (such as amitriptyline and nortriptyline), similar to other neuropathic pain diagnoses.

Your doctor may conduct many tests to diagnose trigeminal neuralgia and determine underlying causes for your condition, including:

• A neurological examination. Touching and examining parts of your face can help your doctor determine exactly where the pain is occurring and — if you appear to have trigeminal neuralgia — which branches of the trigeminal nerve may be affected. Reflex tests also can help your doctor determine if your symptoms are caused by a compressed nerve or another condition.
• Magnetic resonance imaging (MRI). Your doctor may order an MRI scan of your head to determine if multiple sclerosis or a tumor is causing trigeminal neuralgia. In some cases, your doctor may inject a dye into a blood vessel to view the arteries and veins and highlight blood flow (magnetic resonance angiogram).

One published a set of guidelines for diagnosing TN is from the International Headache Society.

For classical TN

• Paroxysmal attacks of pain lasting from a fraction of a second to two minutes, affecting one or more divisions of the trigeminal nerve, and fulfilling criteria 2 and 3.
• Pain has at least one of the following characteristics:
  • Intense, sharp, superficial or stabbing
  • Precipitated from trigger zones or by trigger factors
• Attacks are stereotyped in the individuals patient
• There is no clinically evident neurologic deficit
• Not attributed to another disorder

For symptomatic TN

• Paroxysmal attacks of pain lasting from a fraction of a second to two minutes, with or without persistence of aching between paroxysms, affecting one or more divisions of the trigeminal nerve, and fulfilling criteria 2 and 3.
• Pain has at least one of the following characteristics
  • Intense, sharp, superficial or stabbing
  • Precipitated from trigger zones or by trigger factors.
• Attacks are stereotyped in the individual patient
• A causative lesion, other than vascular compression, has been demonstrated by special investigations and/or posterior fossa exploration.

Treatment of Child Trigeminal Neuralgia

Treatment options include medicines, surgery, and complementary approaches.

Pharmacologic Therapy

• The first-line treatment – for patients with classic TN and idiopathic TN is pharmacologic therapy. The most commonly used medication is the anticonvulsant drug, carbamazepine.[rx] It is usually started at a low dose, and the dose is gradually increased until it controls the pain. It controls pain for most people in the early stages of the disease. However, in some patients, the effectiveness of carbamazepine decreases over time. Possible side effects of carbamazepine include drowsiness, dizziness, double vision, and nausea. In patients with Asian ancestry, before starting carbamazepine, testing for the HLA-B allele is recommended, as its presence increases the risk of development of toxic epidermal necrolysis or Stevens-Johnson syndrome.[rx]

• Oxcarbazepine – is a newer drug and is being increasingly used as first-line therapy for TN in patients who do not respond to or who cannot tolerate carbamazepine (200 to 1200 mg/day) and oxcarbazepine (600 to 1800 mg/day). Possible side effects include double vision and dizziness. It can also cause hyponatremia. It should also be avoided in patients with the HLA-B 15:02 allele.

• Muscle relaxant – Baclofen is a muscle relaxant that can be used to treat TN. Baclofen, lamotrigine, clonazepam, topiramate, phenytoin, gabapentin, pregabalin, and sodium valproate can be used. Side effects include dizziness, sedation, and dyspepsia.

• Seizures, anticonvulsant medication – used to treat epilepsy and to delay or prevent the recurrence of depressive episodes in bipolar disorder. For epilepsy, this includes focal seizures, tonic-clonic seizures, and seizures in Lennox-Gastaut syndrome and medication lamotrigine, phenytoin, gabapentin, clonazepam, and valproic acid. Lamotrigine (200 to 400 mg/day), pregabalin (150 to 600 mg/day), gabapentin (1800 to 4200 mg/day), or topiramate (100 to 400 mg/day) may be considered. If the combination therapy fails, a switch to baclofen (40 to 80 mg/day) may be considered.[rx]

• Eslicarbazepine – an active metabolite of oxcarbazepine, and the new Nav1.7 blocker, vixotrigine, are being explored for pain relief in TN.[rx]

• Anticonvulsant medicines—used to block nerve firing—are generally effective in treating TN1 but often less effective in TN2. These drugs include carbamazepine, oxcarbazepine, topiramate, gabapentin, pregabalin, clonazepam, phenytoin, lamotrigine, and valproic acid.

• Tricyclic antidepressants – such as amitriptyline or nortriptyline can be used to treat pain. Common analgesics and opioids are not usually helpful in treating the sharp, recurring pain caused by TN1, although some individuals with TN2 do respond to opioids.  Eventually, if medication fails to relieve pain or produces intolerable side effects such as cognitive disturbances, memory loss, excess fatigue, bone marrow suppression, or allergy, then surgical treatment may be indicated. Since TN is a progressive disorder that often becomes resistant to medication over time, individuals often seek surgical treatment.

• Botulinum Toxin Injections – This can be beneficial for some patients, particularly the middle-aged and the elderly, who are refractory to medical therapy or who cannot tolerate medical therapy due to their side effects.[rx]

• Tetracaine nerve block – may be used as an additional treatment after carbamazepine, as can acupuncture and/or peripheral nerve stimulation.[rx] Patients with secondary TN also can respond well to pharmacotherapy. However, it is recommended to treat the underlying lesion or disease.

• There is controversy around opiate use such as morphine and oxycodone for treatment of TN, with varying evidence on its effectiveness for neuropathic pain. Generally, opioids are considered ineffective against TN and thus should not be prescribed.^[rx]

Surgical Therapy

• Microvascular decompression – This is one of the most common procedures used to treat trigeminal neuralgia. This is beneficial for patients with TN, where compression of the nerve root is the cause. [rx]This involves craniotomy and posterior fossa exploration for identifying and moving the blood vessel that is compressing the trigeminal nerve. A soft cushion is then inserted between the nerve and the vessel, to allow the nerve to recover, which eventually relieves the pain. In some patients, this procedure can result in sustained pain relief for greater than 10 years. Though this is the most effective procedure, it is also the most invasive one. Some of the complications associated with it are decreased hearing, cerebellar hematoma, CSF leaks, infarction, and facial weakness. It is believed to be the most effective long-term surgical treatment available currently for patients with TN.[rx]
• Ablative procedures include rhizotomy–  with thermocoagulation, chemical injection, or mechanical balloon compression. These procedures involve damaging the trigeminal nerve root, thereby interrupting the pain transmission signals to the brain. Rhizotomy with thermocoagulation uses an electrode to apply heat to damage the nerve fibers. Chemical rhizotomy involves injecting the chemical, glycerol to the trigeminal nerve, thereby damaging it. Balloon compression involves inserting a tiny balloon to the point of location of nerve fibers.[rx][rx] This balloon, on inflation, damages the nerve fibers. Some of the associated complications are postoperative dysesthesia, corneal numbness, sensory loss in trigeminal nerve distribution, and anesthesia Dolorosa.
• Radiosurgery – This procedure involves using radiosurgery instrumentation.[rx][rx][rx] This is a non-invasive procedure, wherein, a highly concentrated dose of ionizing radiation is delivered to a precise target at the trigeminal nerve root. The radiation creates a lesion near the nerve root, thereby interrupting the pain signals from transmission to the brain. The formation of the lesion can be slow, and hence the pain relief using this procedure is delayed by up to several weeks or months. As this is one of the least invasive procedures, it can be repeated in patients who have a recurrence of pain. Some of the associated complications can be facial sensory loss and paresthesias.
• Peripheral neurectomy and nerve block – The neurectomy can be performed on peripheral branches of the trigeminal nerve like the supraorbital, infraorbital, lingual, and alveolar nerves.[rx] This can be accomplished by alcohol injection, incision, cryotherapy, or radiofrequency lesioning. Peripheral neurectomy can be safe in elderly patients in remote and rural areas, where neurosurgical facilities are not readily available.[rx] However, the evidence regarding these peripheral techniques for trigeminal neuralgia is inconclusive.
• A rhizotomy (rhizolysis)–  is a procedure in which nerve fibers are damaged to block pain. A rhizotomy for TN always causes some degree of sensory loss and facial numbness. Several forms of rhizotomy are available to treat trigeminal neuralgia:
• Balloon compression – works by injuring the insulation on nerves that are involved with the sensation of light touch on the face. The procedure is performed in an operating room under general anesthesia. A tube called a cannula is inserted through the cheek and guided to where one branch of the trigeminal nerve passes through the base of the skull. A soft catheter with a balloon tip is threaded through the cannula and the balloon is inflated to squeeze part of the nerve against the hard edge of the brain covering (the dura) and the skull. After about a minute the balloon is deflated and removed, along with the catheter and cannula. Balloon compression is generally an outpatient procedure, although sometimes the patient may be kept in the hospital overnight. Pain relief usually lasts one to two years.
• Glycerol injection – is also generally an outpatient procedure in which the individual is sedated with intravenous medication. A thin needle is passed through the cheek, next to the mouth, and guided through the opening in the base of the skull where the third division of the trigeminal nerve (mandibular) exits. The needle is moved into the pocket of spinal fluid (cistern) that surrounds the trigeminal nerve center (or ganglion, the central part of the nerve from which the nerve impulses are transmitted to the brain). The procedure is performed with the person sitting up, since glycerol is heavier than spinal fluid and will then remain in the spinal fluid around the ganglion. The glycerol injection bathes the ganglion and damages the insulation of trigeminal nerve fibers. This form of rhizotomy is likely to result in the recurrence of pain within a year to two years. However, the procedure can be repeated multiple times.
• Radiofrequency thermal lesioning – (also known as “RF Ablation” or “RF Lesion”) is most often performed on an outpatient basis. The individual is anesthetized and a hollow needle is passed through the cheek through the same opening at the base of the skull where the balloon compression and glycerol injections are performed. The individual is briefly awakened and a small electrical current is passed through the needle, causing tingling in the area of the nerve where the needle tips rest. When the needle is positioned so that the tingling occurs in the area of TN pain, the person is then sedated and the nerve area is gradually heated with an electrode, injuring the nerve fibers.  The electrode and needle are then removed and the person is awakened. The procedure can be repeated until the desired amount of sensory loss is obtained; usually a blunting of sharp sensation, with preservation of touch. Approximately half of the people have symptoms that reoccur three to four years following RF lesioning. Production of more numbness can extend the pain relief even longer, but the risks of anesthesia dolorosa also increase.
• Stereotactic radiosurgery – (Gamma Knife, CyberKnife) uses computer imaging to direct highly focused beams of radiation at the site where the trigeminal nerve exits the brain stem. This causes the slow formation of a lesion on the nerve that disrupts the transmission of sensory signals to the brain. People usually leave the hospital the same day or the next day following treatment but won’t typically experience relief from pain for several weeks (or sometimes several months) following the procedure.  The International Radiosurgery Association reports that between 50 and 78 percent of people with TN who are treated with Gamma Knife radiosurgery experience “excellent” pain relief within a few weeks following the procedure. For individuals who were treated successfully, almost half have a recurrence of pain within three years.
• Decompression Surgery –  is the most invasive of all surgeries for TN, but also offers the lowest probability that pain will return. About half of individuals undergoing MVD for TN will experience recurrent pain within 12 to 15 years.  This inpatient procedure, which is performed under general anesthesia, requires that a small opening be made through the mastoid bone behind the ear. While viewing the trigeminal nerve through a microscope or endoscope, the surgeon moves away from the vessel (usually an artery) that is compressing the nerve and places a soft cushion between the nerve and the vessel. Unlike rhizotomies, the goal is not to produce numbness in the face after this surgery. Individuals generally recuperate for several days in the hospital following the procedure, and will generally need to recover for several weeks after the procedure.
• A neurectomy (also called partial nerve section) – which involves cutting part of the nerve, may be performed near the entrance point of the nerve at the brain stem during an attempted microvascular decompression if no vessel is found to be pressing on the trigeminal nerve. Neurectomies also may be performed by cutting superficial branches of the trigeminal nerve in the face. When done during microvascular decompression, a neurectomy will cause more long-lasting numbness in the area of the face that is supplied by the nerve or nerve branch that is cut. However, when the operation is performed in the face, the nerve may grow back and in time sensation may return.  With neurectomy, there is risk of creating anesthesia Dolorosa.

Surgical treatment for TN2 is usually more problematic than for TN1, particularly where vascular compression is not detected in brain imaging prior to a proposed procedure. Many neurosurgeons advise against the use of MVD or rhizotomy in individuals for whom TN2 symptoms predominate over TN1, unless vascular compression has been confirmed. MVD for TN2 is also less successful than for TN1.

Some individuals manage trigeminal neuralgia using complementary techniques, usually in combination with drug treatment. These therapies offer varying degrees of success. Some people find that low-impact exercise, yoga, creative visualization, aromatherapy, or meditation may be useful in promoting well-being. Other options include acupuncture, upper cervical chiropractic, biofeedback, vitamin therapy, and nutritional therapy. Some people report modest pain relief after injections of botulinum toxin to block the activity of sensory nerves.

Chronic pain from TN is frequently very isolating and depressing for the individual. Conversely, depression and sleep disturbance may render individuals more vulnerable to pain and suffering. Some individuals benefit from supportive counseling or therapy by a psychiatrist or psychologist. However, there is no evidence that TN is psychogenic in origin or caused by depression, and persons with TN require effective medical or surgical treatment for their pain.

References

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