Chronic headaches are headaches that occur for at least 15 days of a month for at least three months. There are a variety of causes and ways to manage this condition. This activity reviews the evaluation and treatment of chronic headache, and explains the role of the interprofessional team in evaluating, treating, managing, and improving care for patients with this condition.
Chronic headache is not a single disease entity but an umbrella term that encompasses all chronic headaches. The International Headache Society defines chronic daily headaches (CDH) as “15 or more headache episodes per month for at least 3 months.”[rx] Chronic headaches are not included as an official class in the International Classification of Headache Disorders (ICHD).[rx]
A chronic daily headache can be divided into primary and secondary headache disorders depending upon its etiology. Primary chronic headache disorders do not have secondary organic etiology. Within the primary headache categories, a headache duration of fewer than 4 hours is labeled as a ‘short headache,’ and more than 4 hours is known as a ‘long headache.’ Long headache is more commonly include chronic migraine and chronic tension headaches.[1] Secondary headaches can occur due to secondary causes such as medication overuse, intracranial tumors, central nervous system (CNS) infections, raised intracranial pressure, metabolic abnormalities, post-traumatic, vascular, and structural pathologies.[rx] It is important to realize that chronic headaches are often caused by a multifactorial combination of the above-mentioned causes and can occur along a continuum.
Causes of Chronic Headaches
The International Classification of Headache Disorders (ICHD) recognizes over 200 headache disorders and divides them into three groups, which are primary, secondary, and painful cranial neuropathies.[rx] The ICHD system is hierarchical with multiple subtypes within each main headache type.
All chronic headaches meet the criteria of occurring at least 15 times a month for at least 3 months, but both primary and secondary chronic headaches have unique characteristics.
Primary headaches lasting greater than four hours include chronic migraines, tension headaches, new daily persistent headaches, and hemicrania continua.
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Chronic migraine has typical migraine features of being unilateral, pulsatile, and moderate to severe and may or may not have an aura.[rx] Episodic migraines may evolve into chronic migraines.
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Chronic migraine in children and adolescents is often bilateral, and associated symptoms such as photophobia and phonophobia are often inferred from behavior.[rx]
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Chronic headaches, which are bilateral, non-pulsatile, and lack associated symptoms, are classified as chronic tension headaches.[rx] Pericranial tenderness is often found on palpation.
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New persistent daily headache (NDPH) occurs suddenly and becomes unremitting within 24 hours of onset. Patients typically have no prior history of headaches. NDPH is rare and refractory to treatment.[rx]
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Hemicrania continua is unilateral, has autonomic symptoms, and is continuous with exacerbations. Responsiveness to indomethacin helps distinguish this form of headache.
Primary headaches lasting less than four hours include chronic cluster headache, neuralgiform headache attacks, and primary stabbing headache.
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Chronic cluster headache varies from the acute form in that there are no remissions, and headaches must occur over at least one year. Headaches are unilateral in the trigeminal distribution and associated with unilateral autonomic symptoms. Patients often experience agitation during the headache.
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The short-lasting neuralgiform headaches include short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). Both types have severe, unilateral pain associated with autonomic symptoms. SUNCT has both lacrimation and conjunctival injection. SUNA may have either but not both of those features and may be accompanied by rhinorrhea or nasal congestion.
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Primary stabbing headaches may frequently occur throughout the day. Sharp, sudden, jabbing pain occurs in the temporal or peri-orbital regions.
Secondary chronic daily headaches include medication overuse, CNS infection, CNS hematomas, intracranial tumor, raised intracranial pressure, low-pressure headache, vasculitis, aneurysms, and cerebrospinal fluid (CSF) leak.[rx]
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Chronic medication overuse headaches often overlap with other acute and chronic headache types. Analgesics are widely used for symptom control in migraine and tension headaches. Patients inadvertently increase headache frequency by overuse of analgesics. The ICHD further classifies this disorder based on the medications used, including NSAIDs, triptans, ergotamines, non-opioid, and opioid analgesics.[rx] Withdrawl of analgesics typically worsens these headaches.
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The remainder of the secondary chronic headache etiologies is beyond the scope of this article.
Diagnosis of Chronic Headaches
A thorough history and physical exam are indispensable in the diagnosis of chronic daily headaches. As noted above, a chronic headache should have 15 or more episodes per month for at least 3 months. One should determine the frequency, intensity, characteristics of the pain, as well as the aggravating and alleviating factors. Many headache types involve ipsilateral autonomic symptoms such as lacrimation, conjunctival injection, conjunctival edema, ptosis, miosis, nasal congestion, rhinorrhea, etc.
A thorough medication reconciliation, including over-the-counter analgesics, is essential. Patients with medication-overuse headaches often have a primary headache disorder, and they frequently use pain medications.[rx] Medication classes may include non-steroidal anti-inflammatory drugs (NSAIDs), triptans, ergotamines, opioids, or a combination of multiple analgesics. Key historical features include morning headaches, the onset of headaches when medication is delayed, and relief when medication is taken.[rx]
Comorbidities, sleep history, and family history of headaches should also be noted. A secondary headache disorder should be excluded from the history and examination.
Recognition of headache “red flags” is a critical piece in identifying secondary headaches and ordering additional diagnostic testing. Those “red flags” include:[rx]
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Age above 50
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Significant change in prior headache pattern
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Severe, “thunderclap” headache
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Systemic illness signs such as fever
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Known illness which increases the risk for secondary headaches such as cancer or HIV
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Neurologic symptoms
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Headaches associated with Valsalva maneuvers
Physical exam findings concerning secondary headache causes include focal neurological deficits, papilledema, bitemporal hemianopia, homonymous hemianopia, decrease visual acuity or increased pain with the Valsalva method.
Primary chronic headaches often lack physical findings but may have autonomic activation or muscle tenderness in the occipital or cervical regions.
Evaluation
In a straightforward chronic primary headache disorder, further evaluation may not be warranted, but many clinicians will advise for baseline laboratory testing and brain imaging to exclude the secondary treatable causes.
Laboratory workup includes a complete blood count to look for infection. Erythrocyte sedimentation rate (ESR) is increased in giant cell arteritis and other vasculitides. A metabolic panel to look for metabolic causes of headache, and endocrine testings to look for pituitary gland abnormalities.
Magnetic resonance imaging (MRI) of the brain is the imaging modality of choice.[rx] A contrast study is often recommended to increase the sensitivity and specificity to detect structural abnormalities. A need for vascular imaging is based on the differential diagnosis. Further studies may be warranted depending upon the underlying cause. These may include positron emission tomography (PET) scan, magnetic resonance spectroscopy (MRS), and/or biopsy. A lumbar puncture may be required if there is suspicion of a CNS infection or idiopathic intracranial hypertension.
Treatment of Chronic Headaches
Treatment and management of chronic headache disorders depend upon the underlying etiology and may require an interprofessional approach.
A patient should maintain a headache journal that will document their headache episodes and any accompanying triggers. If found, stressors should be avoided or minimized.
Chronic Migraine
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Chronic migraine treatment should begin with setting the expectation that headache frequency and severity will decrease, but headaches will not be eliminated.
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The patient should be counseled that high caffeine intake, sleep deprivation, overuse of analgesics, and comorbid conditions can worsen chronic migraines.
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Prophylactic pharmacologic treatment should be used. First-line therapy includes beta-blockers, anticonvulsants, and antidepressants. The most commonly used medications are propranolol, topiramate, and amitriptyline.[rx]
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Botulinum toxin A is a Food and Drug Administration (FDA) approved treatment for chronic migraines and is considered second-line therapy.
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Monoclonal antibodies that target calcitonin gene-related peptides (CGRP) are the newest development in chronic migraine treatment. Erenumab, fremanezumab, and galcanezumab are approved for chronic migraines, which have failed to respond to other treatments[rx].
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Triptans, steroids, NSAIDs, and opioids are often used to abort acute episodes, but routine use of these medications increases the risk of developing a medication-overuse headache.
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Patients may also benefit from psychological counseling if anxiety or depression is present.
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Manual medicine, such as spinal manipulation and trigger point treatment, may be used as a complementary or alternative therapy.[rx]
Chronic Tension Headache
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Amitriptyline, a tricyclic antidepressant, is recommended as the first-line treatment for chronic tension headaches.
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Amitriptyline, in addition to inhibiting the reuptake of serotonin and noradrenaline, also reduces tenderness in pericranial muscles.
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Tricyclic antidepressants increase the risk for cardiac arrhythmia, and patients should be screened for cardiovascular disorders prior to initiating therapy. Patients over 40 should undergo an ECG.
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Anticonvulsants, such as topiramate and gabapentin, can be considered as second-line treatment.
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Addressing the potential musculoskeletal causes of tension headache, treatment with physical therapy, acupuncture, trigger point injections, spinal manipulation, or muscle relaxants may be beneficial.[rx]
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Behavioral therapy, including cognitive-behavioral therapy, biofeedback, and relaxation techniques, is particularly helpful for patients with coexisting anxiety or depression.
Medication Overuse Headache
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Patient education about the potential for overuse of analgesic medication to lead to headache progression is key. Include the use of over-the-counter analgesics in the discussion.
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The physician initiates a preventative medication while simultaneously assisting the patient in discontinuing the causative medication.
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Patients may experience withdrawal symptoms of nausea and anxiety for 2 to 10 days when the analgesic medication is discontinued.
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There is no consensus on the most appropriate medication for use as bridge therapy following discontinuation of the offending drug. Long-acting NSAIDs, prednisone, dihydroergotamine, and antiemetics are options.[24] The medication should not be from the same class as the offending medication.
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Medications that may be effective for prophylaxis include topiramate, amitryptiline, valproic acid, and beta-blockers. The choice of medication should be based upon comorbidities and the primary headache disorder.[25]
Chronic Autonomic Cephalgia
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Indomethacin is the drug of choice for paroxysmal hemicrania, hemicrania continua, primary stabbing headache, hypnic headache, and Valsalva-induced headaches (e.g., cough headache, exercise headache).
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Verapamil is the drug of choice for the prevention of chronic cluster headaches. Verapamil requires titration to become effective, and glucocorticoids or dihydroergotamine can be used for exacerbations.
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Chronic cluster headaches not responsive to pharmacologic therapy can be treated with a non-invasive vagus nerve stimulator or sphenopalatine ganglion microstimulator.[rx]
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First-line prophylactic therapy for chronic SUNCT and SUNA is lamotrigine. Topiramate and gabapentin are alternatives.[rx]
Alternative treatment
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Changes in lifestyle; must be a commitment from the patient; however, social support is of great importance to improve mental health to help the patient’s involvement.
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Regular exercise
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Yoga
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Relaxation training
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Cognitive-behavioral therapy
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Biofeedback
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Reduction of triggers
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Detoxification
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Butterbur
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Melatonin
Children and Headache
Headaches are common in children. Headaches that begin early in life can develop into migraines as the child grows older. Migraines in children or adolescents can develop into tension-type headaches at any time. In contrast to adults with migraines, young children often feel migraine pain on both sides of the head and have headaches that usually last less than 2 hours. Children may look pale and appear restless or irritable before and during an attack. Other children may become nauseous, lose their appetite, or feel pain elsewhere in the body during the headache.
Headaches in children can be caused by a number of triggers, including emotional problems such as the tension between family members, stress from school activities, weather changes, irregular eating and sleep, dehydration, and certain foods and drinks. Of special concern among children are headaches that occur after a head injury or those accompanied by rash, fever, or sleepiness.
It may be difficult to identify the type of headache because children often have problems describing where it hurts, how often the headaches occur, and how long they last. Asking a child with a headache to draw a picture of where the pain is and how it feels can make it easier for the doctor to determine the proper treatment.
Migraine in particular is often misdiagnosed in children. Parents and caretakers sometimes have to be detectives to help determine that a child has a migraine. Clues to watch for include sensitivity to light and noise, which may be suspected when a child refuses to watch television or use the computer, or when the child stops playing to lie down in a dark room. Observe whether or not a child is able to eat during a headache. Very young children may seem cranky or irritable and complain of abdominal pain (abdominal migraine).
Headache treatment in children and teens usually includes rest, fluids, and over-the-counter pain relief medicines. Always consult with a physician before giving headache medicines to a child. Most tension-type headaches in children can be treated with over-the-counter medicines that are marked for children with usage guidelines based on the child’s age and weight. Headaches in some children may also be treated effectively using relaxation/behavioral therapy. Children with cluster headaches may be treated with oxygen therapy early in the initial phase of the attacks.
Headache and Sleep Disorders
Headaches are often a secondary symptom of a sleep disorder. For example, tension-type headache is regularly seen in persons with insomnia or sleep-wake cycle disorders. Nearly three-fourths of individuals who suffer from narcolepsy complain of either migraine or cluster headache. Migraines and cluster headaches appear to be related to the number of and transition between rapid eye movement (REM) and other sleep periods an individual has during sleep. Hypnic headache awakens individuals mainly at night but may also interrupt daytime naps. Reduced oxygen levels in people with sleep apnea may trigger early morning headaches.
Getting the proper amount of sleep can ease headache pain. Generally, too little or too much sleep can worsen headaches, as can the overuse of sleep medicines. Daytime naps often reduce deep sleep at night and can produce headaches in some adults. Some sleep disorders and secondary headaches are treated using antidepressants. Check with a doctor before using over-the-counter medicines to ease sleep-associated headaches.
Coping with Headache
Headache treatment is a partnership between you and your doctor, and honest communication is essential. Finding a quick fix to your headache may not be possible. It may take some time for your doctor or specialist to determine the best course of treatment. Avoid using over-the-counter medicines more than twice a week, as they may actually worsen headache pain and the frequency of attacks. Visit a local headache support group meeting (if available) to learn how others with headaches cope with their pain and discomfort. Relax whenever possible to ease stress and related symptoms, get enough sleep, regularly perform aerobic exercises, and eat a regularly scheduled and healthy diet that avoids food triggers. Gaining more control over your headache, stress, and emotions will make you feel better and let you embrace daily activities as much as possible.
What Research is Being Done?
Several studies either conducted or supported by the National Institute of Neurological Disorders and Stroke (NINDS), a part of the National Institutes of Health, are revealing much about the headache process and may lead to new treatments or perhaps ways to block debilitating headache pain. Studies by other investigators are adding insight to headache etiology and treatment.
Understanding headache mechanisms and underlying causes
The molecular basis for migraine headaches and the aura associated with certain migraines is uncertain. One multi-faceted research study is examining how migraine with aura may affect metabolism and neurophysiological function. Investigators are also studying if particular regions of the visual cortex are unusually susceptible to the events in the brain that cause the aura. Another study component is investigating what happens at the beginning of a headache and how changes in the brain’s meninges may lead to vascular and trigeminal nerve stimulation associated with the painful part of a migraine headache. Results may provide a greater understanding of migraine and assist the development of new therapies.
Mast cells, which are part of the immune system and are involved in the inflammatory allergic response, are activated in some chronic pain conditions, including headache. Researchers are examining the possibility of a relationship between the mast cells’ anti-analgesic properties and their proximity to and enhanced activation of nerve fiber endings that receive and transmit pain signals (nociceptors). Mast cells may release substances that activate nociceptive nerve cells that transmit signals from the linings of the skull and its blood vessels. Findings that link mast cell activation to headache pain may identify drug targets that could lead to new analgesics for headache and other pain syndromes.
Cortical spreading depression (CSD) is a process in migraine with aura in which a wave of increased brain activity, followed by decreased activity, slowly spreads along the brain’s surface. The wave of brain activity often travels across the part of the brain that processes vision and corresponds to the typical visual aura of migraine. Research has shown that migraines with aura may be associated with tiny areas of stroke-like brain damage caused by a short-term drop in oxygen levels (associated with the CSD) which prevents normal cell function and swelling in the brain’s nerve cells. Animal studies have shown that CSD also irritates the trigeminal nerve, causing it to transmit pain signals and trigger inflammation in the membranes that surround the brain. CSD inhibiting drugs such as tonabersat are being tested in clinical trials for their usefulness in treating migraine and other neurological diseases. Other investigators hope to build on initial results showing that estrogen withdrawal makes it easier for CSD to occur in the brains of animals, which may explain the contribution of estrogen fluctuation to menstrual migraines. This research may result in a better understanding of how a migraine starts in the brain and offer new methods of treatment by interrupting this process and preventing the migraine.
Cutaneous allodynia is the feeling of pain or unpleasant sensations in response to normally nonpainful stimuli, such as light touch. Researchers are investigating why it is present on the head or face in people with cluster headaches, to better understand neurological changes that occur with these headaches. Similar research is looking at why some people with migraines have more than the typically restricted allodynia that affects a particular area of the head predicted by the headache (for example, on the same side of the face as the migraine pain). Individuals with extended allodynia may experience unpleasant sensations on the side of the face opposite the headache pain or even on their feet. Previous studies have shown that sensitized nociceptors in the brain’s coverings are involved in the throbbing pain of migraine and that other sensitized neurons found deeper in the brain are involved with restricted allodynia, but it is not certain which cells are responsible for extended allodynia. Future studies will explore whether nerve cells in the thalamus (which is involved in relaying signals between the brain and the body) become more sensitive a result of headache pain and cause extended allodynia. Findings may offer a better understanding of how the nervous system changes and becomes more sensitive after repeated stimulation, resulting in chronic pain.
Social and other factors may impact headaches. Researchers are examining how race and psychiatric conditions are related to headache severity, quality of life, the ability to reliably follow a treatment program, and treatment response in people with migraines, tension-type headache, substance abuse headache, or cluster headache.
Genetics of headache
Genetics may contribute to a predisposition for migraines. Most migraine sufferers have a family member with migraine. Researchers are studying the activity of different genes to see if they make some people more likely to have migraines. One strategy is to test for a gene in several families having members with migraines and then determine if the gene is related to migraine in a broader population.
In April 2008, researchers at the University of Helsinki reported significant evidence for linkage between a gene variant on a specific site on chromosome 10q22-q23 and susceptibility to common types of migraine. The findings were from a study of 1,675 migraine sufferers or their close relatives from 210 Finnish and Australian migraine families. Another study replicated the findings in the two populations and also showed that the site was particularly linked to female migraine sufferers. Although it has been known for some time that genetic factors shared by family members make people more susceptible to migraines, this study is the first to identify convincingly a specific gene locus for common forms of migraine.
Currently under investigation are gene expression patterns (signs of changes in gene activity) in the blood of individuals during migraine attacks and among individuals with chronic daily headaches. Preliminary studies show that children with acute migraines and chronic daily headaches have specific similar gene expression profiles in their blood that are different from healthy individuals and from children with other non-related neurological diseases. Researchers are exploring differences in gene expression profiles among individuals who respond to different types of headache drugs. Study results may indicate a molecular genomic approach using blood samples to detect genes that may be activated during headaches and identify which drugs are best used for each person with migraines.
Scientists are exploring the role of the calcitonin gene-related peptide (CGRP) in migraines. Levels of the CGRP molecule, which is involved in sending signals between neurons, increase during migraine attacks and revert to normal when the pain resolves. Researchers plan to use CGRP as a model and then to use functional magnetic resonance imaging to estimate the pain response in the central nervous system. Evidence from individuals with Familial Hemiplegic Migraine (FHM) with known mutations indicates that migraine pathways in FHM may be different from normal migraine. Investigators are also measuring levels of CGRP during the premonitory, mild, moderate, and severe phases of a single migraine compared to the baseline level when individuals are pain-free. The fluctuations of CGRP during the migraine process will help to define its role in migraine pain and may offer new opportunities for acute treatment.
Clinical studies in headache management
A major focus of headache research is the development of new drugs and other treatment options. Several drug studies seek to identify new drugs to treat various headache disorders and to find safer, more effective doses for medications already being used. Other research is aimed at identifying receptors or drug targets to stop the process of migraine aura in the brain.
Results of three randomized, placebo-controlled clinical trials show the drug topiramate is effective, safe, and generally well-tolerated for treating chronic migraine. Experts agree that treatment with combinations of preventive agents offers maximum relief for the majority of individuals with chronic migraines. An NINDS-funded clinical trial is examining the effectiveness and safety of the drug propranolol combined with topiramate in reducing the frequency of chronic migraine in 250 participants who will be randomly selected to receive treatment with both drugs or topiramate and placebo.
Sleep plays an important role in migraine. Migraine in older adults is sometimes triggered by sleep changes; regulating their sleep may lessen the frequency of migraines. Younger migraine sufferers often report migraine relief after sleep. Researchers are studying the use of the drug ramelteon, which is approved by the U.S. Food and Drug Administration for insomnia, in reducing the number of migraines over a 12-week period.
Headache is the most common symptom after a closed head injury, and it can last for more than 2 months in 60 percent of affected individuals. Unfortunately, individuals with chronic post-traumatic headaches also have cognitive and behavioral problems, and many drugs currently used to treat the headaches also have a negative influence on cognition. Scientists are testing different drugs, such as naratriptan (which acts as a neurotransmitter) and galantamine (used to treat Alzheimer’s disease), to treat both headache and cognitive disturbances in individuals with chronic post-traumatic headaches.
Non-pharmaceutical approaches to treatment and prevention
Historically, very little research has been done on children with headaches. A variety of headache education and drug and/or behavioral management techniques are aimed at improving headache treatment and prevention in children and adolescents. Scientists are testing the effectiveness of combined pain coping skills (including age-appropriate biofeedback, muscle relaxation techniques, imagery, activity pacing, and the use of calming techniques) and the drug amitriptyline in reducing headache frequency, intensity, and depressive symptoms in youth ages 10 to 17 years. Additional studies include the use of alternative approaches such as yoga to decrease headaches in adolescents, a modified diet to treat chronic daily headaches in teenagers, and programs designed to teach very young children how to understand and self-manage their headaches.
Craniosacral therapy (CST) involves gentle massaging of the neck, head, and spine to release constraints in tissue in the head and around the spine. Limited preliminary data shows significant, the sustained benefit of CST in a small group of individuals with migraines. Future research will gather data on the usefulness of CST in preventing migraines and examine the feasibility of a larger, randomized trial.
Electrical stimulation of the occipital nerve has effectively eased the symptoms of painful chronic headache conditions such as cluster headache as well as hard-to-treat migraine in small clinical studies. A tiny battery-powered rechargeable electrode, surgically implanted near the occipital nerve, sends continuous energy pulses to the nerve to ease pain. The use of this non-drug treatment in reducing migraine frequency, intensity, and effect on the quality of life is being tested in larger clinical trials.
References
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