Abstract
Headaches are a very common and potentially disabling neurologic condition that can significantly impact the quality of life. There are three major primary headache disorders: migraine, cluster, and tension-type headache, and they are differentiated based on clinical criteria outlined in the International Classification of Headache Disorders. The key to optimizing treatment is identifying the appropriate headache phenotype with a detailed history, physical, and neurologic examination. Headache treatment can consist of both non-pharmacologic and pharmacologic treatment measures. Non-pharmacologic treatment includes lifestyle modifications, physical therapy, cognitive-behavioral therapy, biofeedback-assisted relaxation, or acupuncture. Pharmacologic approaches to management of headache pain include oral medications, which can be abortive and/or prophylactic. For resistant headaches, injections or surgery may be considered.
Keywords
Cluster headache, headache, migraine, migraine headaches, tension-type headache
Synonyms | |
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ICD-10 Codes | |
G44.209 | Tension-type headache, unspecified, not intractable |
G43.909 | Migraine, unspecified, not intractable, without status migrainosus |
R51 | Headache |
G44.029 | Chronic cluster headache, not intractable |
Definition
The three major primary headache disorders are migraine, cluster, and tension-type headache. Although all three syndromes are characterized by chronic, recurrent, and potentially disabling headaches, specific diagnosis is important because of differing natural history and treatment.
Headache disorders are classified according to criteria outlined in the International Classification of Headache Disorders (ICHD), originally developed by the International Headache Society in 1988 and revised in 2004 with a third revision recently reviewed as a “beta” version in 2013 and finalized in 2018 as the ICHD-3. According to the ICHD, the third edition of headache criteria (referred to as ICHD-3) was released as a beta version available to all for public scrutiny and community input on how these criteria will align with recent revision to the International Classification of Diseases-11 by the World Health Organization. The criteria have now been formally adopted and are available online ( www.ihs-headache.org ; www.ichd-3.org ). Changes to criteria for the three primary headache disorders are relatively minor under ICHD-3.
Diagnosis of all but a few rare migraine subtypes remains clinical, based on the patient’s history and an examination that rules out secondary causes of headache (not covered in this chapter). The ICHD criteria were developed for research purposes and therefore may lack sensitivity when they are used in the clinical setting. Both migraine and tension-type headaches are more common in women than in men, while cluster headache is generally a male disorder. Peak prevalence of migraine occurs during midlife, when it affects almost a quarter of all women and roughly 10% of men. Recurrent headaches are not rare in children, but accurate diagnosis can be difficult. Headache presentation in children varies from that in adults, and children may have difficulty describing the headache characteristics needed to differentiate between the subtypes.
Migraine
Migraine is subclassified as migraine without aura ( Table 103.1 ) and migraine with aura ( Table 103.2 ). Under ICHD-3, “chronic migraine,” which was previously classified as a migraine complication, is now also included as a migraine subtype. Individuals with headaches occurring 15+ days per month for 3 or more months and who report migraine symptoms for 8 or more days per month would qualify for a chronic migraine diagnosis. According to ICHD-3 , chronic migraine was included as a subtype because “it is impossible to distinguish the individual episodes of headache in patients with such frequent or continuous headaches,” (p. 650). About 20% of patients have aura, usually preceding the headache, which consists of focal neurologic signs or symptoms that begin gradually and fade away within 30 to 60 minutes as the headache begins. The most common type of aura involves visual disturbances, typically an enlarging scotoma, but patients can also see shapes such as stars, zigzag lines, or other visual distortions, including field cuts and photopsias. However, blurred vision is usually not considered a part of aura. Sensory or motor problems occur far less frequently. Migraine can also be classified as episodic or chronic (15 or more migraine headache days per month). With diligent record tracking, some patients are able to identify triggers for their headache, including exertion, certain foods, scents, and hormonal influences. Three gene mutations have been identified that are associated with a particular subtype of migraine with aura known as familial hemiplegic migraine. These genes influence the stability of neuronal cell membranes.
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Many patients with migraine report prodromal symptoms: yawning; neck and shoulder muscle discomfort; excessive salivation; and changes in appetite, mood, sleep, gastrointestinal function, and urination. Some of these can start days before the actual headache. Postdromal symptoms in migraine include fatigue, exercise intolerance, and neck and shoulder muscle discomfort. If headaches progress untreated, 80% of patients eventually develop allodynia, defined as pain in response to stimuli that normally are nonpainful. Once allodynia develops, treatment may be less successful. In addition, there is increasing attention on cognitive symptoms of migraine that likely contribute to disability. Pain-related disability is a well-recognized target of migraine treatment, so the assessment of migraine-related cognitive symptoms is recommended.
Cluster
Cluster headaches are strictly unilateral headaches that are far more common in men than in women, but the prevalence in the general population overall is approximately 0.1%. The pain is sharp and steady, in contrast to the throbbing pain of migraine, and localized to the orbital area. Diagnostic criteria require the presence of at least one autonomic sign or symptom during the headache, such as ipsilateral conjunctival injection, lacrimation, rhinorrhea, ptosis, or miosis.
Cluster headache is so called because the headaches occur regularly in most cases, from one to eight times a day, during a period of 2 weeks to 3 months that is referred to as a cluster episode. The headaches then completely remit for months or years. In chronic cluster headache, there are no headache-free periods or remissions, or they are less than 2 weeks in duration. Patients with cluster headache usually describe alcohol intolerance during the cluster episode and generally note intense restlessness during the headache.
Tension Type
Tension-type headaches can vary in length from 30 minutes to 7 days. Diagnostic criteria for tension-type headaches are largely unchanged under ICHD-3. They are typically bilateral, moderate in intensity, and described as a pressing, squeezing sensation that is not affected by physical activity. Associated symptoms, such as nausea, vomiting, photophobia, and phonophobia, are generally not present or are mild. Tension-type headache is subclassified as infrequent episodic if it occurs less than 1 day per month, frequent episodic if it occurs from 1 day to 14 days per month, and chronic with attacks occurring 15 days or more per month for at least 6 months. Patients with tension-type headache typically do not spontaneously report symptoms other than headache. A diagnosis of migraine should be reexamined if multiple associated symptoms are reported, like nausea and photophobia, which are thought to be a part of the sympathetic activation that occurs with migraines.