Chapter 24 - Migraine: clinical diagnostic criteria

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Migraine headache represents a diverse group of disorders that includes migraine with and without aura, childhood periodic syndromes, retinal migraine, complications of migraine, and probable migraine. The criteria as proposed by the International Headache Society (IHS) have standardized the diagnosis of migraine headache for use within clinical and research settings. The IHS diagnostic criteria have employed a hierarchical structure which divides all headaches into two broad categories: secondary headache disorders, in which the headache may be attributed to an identifiable underlying abnormality (etiological diagnosis), and primary headache disorders, in which such a causal abnormality cannot be identified (syndromic or symptomatic diagnosis). The primary and secondary headache disorders are divided into headache types, subtypes, and subforms. Such a hierarchical system allows physicians and scientists to employ the criteria to the level of precision required for their purposes. Further research in the field will guide future revisions of the diagnostic criteria to provide more precise and accurate diagnoses of this complex disorder called “migraine headache.”

Introduction

The criteria formulated by the International Headache Society (IHS), first in 1988 (Headache Classification Committee of the International Headache Society, 1988) and then revised and published in a second edition in 2004 (Headache Classification Subcommittee of the International Headache Society, 2004), are widely accepted as the basis for accurate clinical diagnosis of headache disorders. Over the past 18 years the IHS criteria have been very helpful in defining homogeneous populations for clinical research and have gradually had an important beneficial effect on daily clinical practice. Both editions of the IHS diagnostic criteria have employed a hierarchical structure which divides all headaches into two broad categories: secondary headache disorders, in which the headache may be attributed to an identifiable underlying abnormality (etiological diagnosis), and primary headache disorders, in which such a causal abnormality cannot be identified (syndromic or symptomatic diagnosis). The primary and secondary headache disorders are then, in turn, divided into headache types, subtypes, and subforms. Such a hierarchical system allows physicians and scientists to employ the criteria to the level of precision required for their purposes.

In the case of migraine, the disorder as a whole is coded as 1 based on the characteristics of the acute headache attack. The two major subtypes of migraine are then divided into 1.1 migraine without aura and 1.2 migraine with aura, based on the presence or absence of transient focal neurological symptoms. Migraine with aura is then further subdivided into 1.2.1 typical aura with migraine headache, 1.2.2 typical aura with non-migraine headache, and 1.2.3 typical aura without headache, based on the co-occurrence of focal neurological symptoms with headache. Other subforms of aura include 1.2.4 familial hemiplegic migraine (formerly motor aura) and 1.2.5 sporadic hemiplegic migraine, derived from new information about the genetic underpinnings of motor symptoms that can accompany migraine headache, as well as 1.2.6 basilar-type migraine, based on the presumed brainstem (non-cortical) origin of the neurological symptoms. Other subtypes of migraine include: 1.3 childhood precursors of migraine; 1.4 retinal migraine, in which visual disturbance is monocular (prechiasmal) rather than homonymous (retrochiasmal); 1.5 complications of migraine; and 1.6 probable migraine, in which the headache fails to fulfill one of the criteria required for definite diagnosis.

Because the criteria for the primary headache disorders are based on the symptom characteristics of the acute attack, the criteria for migraine and the other primary headache disorders always include the final caveat: “not attributable to another disorder.” The diagnostic criteria for migraine remained fairly stable from the first edition to the second edition, with the most prominent changes including the addition of criteria for the diagnosis of chronic migraine and the separate categorization of hemiplegic migraine. The criteria for migraine are dynamic and likely to show continuing change over the coming decades as our knowledge of migraine genetics and pathophysiology evolves.

Section snippets

Migraine Without Aura

Migraine without aura is defined by the specific characteristics of the headache attack, its duration, and the associated symptoms that accompany it. IHS criteria (the second edition of the International Classification of Headache Disorders (ICHD-II): Headache Classification Subcommittee of the International Headache Society, 2004) require that an individual have five past attacks that fulfill the following criteria:

  • Duration of 4–72 h

  • Two of more of the following headache characteristics:

Migraine with Typical Aura

The IHS criteria define migraine with aura as a “recurrent disorder manifesting in attacks of reversible focal neurological symptoms that usually develop gradually over 5–20 minutes and last for less than 60 minutes.” Headache with the features of migraine without aura usually follows the aura symptoms. Less commonly, headache lacks migrainous features or is completely absent (Headache Classification Subcommittee of the International Headache Society, 2004).

To fulfill the IHS diagnostic

Familial and Sporadic Hemiplegic migraine

The availability of new genetic data regarding patients with transient focal motor symptoms has allowed a more precise diagnosis of hemiplegic migraine than was available in the past. As a result hemiplegic migraine was separated as a distinct diagnostic category from the other typical forms of migraine with aura. Hemiplegic migraine has been further subdivided into two forms: familial hemiplegic migraine (FHM) (1.2.4), in which a patient has motor aura and a first- or second-degree relative

Basilar-type migraine

The IHS criteria recognize a distinct subtype of migraine aura in which the aura symptoms originate from brainstem and/or bilateral cerebral hemispheric sources and are not associated with motor symptoms. Basilar-type symptoms frequently occur in FHM1. So the presence of motor symptoms, even if other symptoms suggestive of basilar-type migraine are present, would require the diagnosis of hemiplegic migraine (1.2.4 or 1.2.5) rather than basilar-type migraine (1.2.6).

To fulfill the IHS diagnostic

Childhood periodic syndromes

Three syndromes described in children are recognized in the IHS criteria as precursors to migraine. Small population-based studies suggest a relationship between migraine and these disorders (Abu-Arafeh 1995a, Abu-Arafeh, 1995b, Abu-Arafeh, 1995c).

Cyclical vomiting (1.3.1) is a self-limited condition of childhood consisting of episodes of severe nausea, pallor, and lethargy separated by symptom-free periods. During attacks the child may also experience photophobia, phonophobia, and exercise

Retinal Migraine

Retinal migraine refers to a monocular visual impairment that occurs in temporal association with a headache that meets diagnostic criteria for migraine without aura. The monocular visual impairment generally represents a visual loss, but scotomata, flashing lights, haloes, and zigzag lines have been reported. The symptoms can last for seconds, days, or even weeks and may recur repeatedly in the same patient. The pathophysiology is unknown, but may occur as a result of vasospasm of ciliary or

Chronic Migraine

Chronic migraine was a new addition to the revised IHS criteria published in 2004. A diagnosis of chronic migraine requires (Headache Classification Subcommittee of the International Headache Society, 2004):

  • ≥15 days of headache per month that fulfills criteria for migraine without aura for longer than 3 months

  • Not attributable to another disorder.

These criteria have been controversial for several reasons. First, chronic migraine often evolves from a less frequent episodic pattern and, as the

Status Migrainosus

Status migrainosus refers to the persistence of an attack of migraine without aura beyond the 72 h typically encountered with episode attacks. The headache attack also has to be severe and unremitting over that time period and not attributed to another disorder. Less severe migraines that persist beyond 72 h would be classified as probable migraine (see below) (Headache Classification Subcommittee of the International Headache Society, 2004).

Persistent aura without infarction

If a patient experiences persistence of a typical previously transient aura symptom for greater than 60 min and neuroimaging demonstrates no cerebral infarction in the relevant brain area, then the patient is considered to have persistent aura without infarction (1.5.3). This is a rare condition with no known reliably effective treatment. It should be differentiated from migrainous infarction and posterior leukoencephalopathy (Headache Classification Subcommittee of the International Headache

Migrainous infarction

A migrainous infarction is diagnosed when a patient has an aura typical of a past attack that lasts longer than >60 min and a cerebral infarction is noted on neuroimaging in the relevant brain area (Headache Classification Subcommittee of the International Headache Society, 2004). This could account for 0.5–1.5% of all strokes or 10% of strokes in younger patients (Sacquegna et al., 1989, Arboix et al., 2003). The pathophysiology of migrainous infarction is unknown, but has been postulated to

Migraine-triggered Seizures

A diagnosis of migraine-triggered seizures is established in a patient who meets IHS criteria for migraine with aura (1.2) and seizure occurs during or within 1 h after a migraine aura (Headache Classification Subcommittee of the International Headache Society, 2004). Marks and Ehrenberg (1993) identified 395 patients who experienced both migraine as well as a seizure disorder and reported that 3% of these patients experienced a seizure during or immediately after an aura. The pathophysiology

Probable Migraine

Probable migraine refers to attacks and/or headache that fulfill all but one of the diagnostic criteria for a subtype of migraine headache (Headache Classification Subcommittee of the International Headache Society, 2004). Probable migraine is categorized into three subtypes: probable migraine without aura (1.6.1), probable migraine with aura (1.6.2), and probable chronic migraine (1.6.5). Epidemiological studies from France have reported that 11.2% of the general population meet strict

Conclusion

Migraine headache represents a diverse group of disorders that includes migraine with and without aura, childhood periodic syndromes, retinal migraine, complications of migraine, and probable migraine. The criteria as proposed by the IHS have standardized the diagnosis of migraine headache for use within clinical and research settings. Controversy exists with some of the proposed criteria (particularly with migraine without aura and chronic migraine), which only serves to emphasize the fact

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