KEY POINT: MIGRAINE: SPECTRUM A Most patients develop migraine in the first 3 OF SYMPTOMS decades of life, some in the AND DIAGNOSIS fourth and even the fifth decade. William B. Young, Stephen D. Silberstein ABSTRACT The migraine attack can be divided into four phases. Premonitory phenomena occur hours to days before headache onset and consist of psychological, neuro- logical, or general symptoms. The migraine aura is comprised of focal neurological phenomena that precede or accompany an attack. Visual and sensory auras are the most common. The migraine headache is typically unilateral, throbbing, and aggravated by routine physical activity. Cutaneous allodynia develops during un- treated migraine in 60% to 75% of cases. Migraine attacks can be accompanied by other associated symptoms, including nausea and vomiting, gastroparesis, di- arrhea, photophobia, phonophobia, osmophobia, lightheadedness and vertigo, and constitutional, mood, and mental changes. Differential diagnoses include cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoenphalopathy (CADASIL), pseudomigraine with lymphocytic pleocytosis, ophthalmoplegic mi- graine, Tolosa-Hunt syndrome, mitochondrial disorders, encephalitis, ornithine transcarbamylase deficiency, and benign idiopathic thunderclap headache. Migraine is a common episodic head- (Headache Classification Subcommittee, ache disorder with a 1-year prevalence 2004): of approximately 18% in women, 6% inmen,and4%inchildren.Attacks Recurrent attacks of headache, consist of various combinations of widely varied in intensity, fre- headache and neurological, gastrointes- quency, and duration. The attacks tinal, and autonomic symptoms. Most are commonly unilateral in onset; patients develop migraine in the first are usually associated with an- 67 3 decades of life, some in the fourth orexia and sometimes with nausea and even the fifth decade. The term and vomiting; some are preceded migraine is derived from the Greek by, or associated with, conspicu- word hemicrania, introduced by Galen ous sensory, motor, and mood dis- in approximately AD 200. turbances; and are often familial. In order to increase precision, the DIAGNOSIS International Headache Society (IHS) Early migraine descriptions stressed proposed and published its classifica- three features: the unilateral distribu- tion of headache disorders in 1988 and tion of the headache, the presence of revised it in 2004 (Headache Classifi- a warning (often visual), and nausea or cation Subcommittee, 2004). The IHS vomiting. The Ad Hoc Committee on criteria added photophobia and pho- Classification of Headache described nophobia and required an untreated vascular headache of migraine type as duration of 4 to 72 hours. Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited. " MIGRAINE: SYMPTOMS AND DIAGNOSIS KEY POINTS: A Migraine headaches were formerly To establish a diagnosis of migraine At most, only divided into two varieties: classic and under the IHS classification, certain 30% of common, which are now called mi- clinical features must be present and migraineurs have classic graine with aura and migraine without organic disease must be excluded aura. The same aura. The aura is the complex of focal (Table 4-2). Several attacks, each patient may neurological symptoms that precedes lasting 4 to 72 hours (untreated), are have migraine or accompanies an attack. At most, necessary and must have two of the headache only 30% of migraineurs have ‘‘classic’’ following four pain characteristics: without aura, aura. The same patient may have unilateral location, pulsating qual- migraine migraine headache without aura, mi- ity, moderate to severe intensity, and headache with graine headache with aura, and mi- aggravation by routine physical activ- aura, and graine aura without headache. ity. In addition, the attacks must have migraine aura In addition to the symptoms de- at least one of the following associated without scribed in the IHS definition, other symptoms: nausea and/or vomiting, headache. symptoms of migraine are extremely and/or photophobia and phonopho- A If sufficient common. A wide variety of premoni- bia. Using these criteria, no single criteria but one tory symptoms may occur; nasal con- associated feature is mandatory for are present, the gestion and neck pain may be present; diagnosing migraine, although recur- headache is and the typical eye findings of cluster rent episodic attacks must be docu- called probable headache may occur with some regu- mented. Different patients or different migraine. larity (Table 4-1). attacks may fulfill entirely nonover- lapping criteria. If sufficient criteria but one are present, the headache is TABLE 4-1 Rates of Migraine called probable migraine. Table 4-2 Symptoms lists the current subtypes of migraine. A migraine attack usually lasts less Symptom Approximate % than a day; when it persists for more * than 3 days, the term status migrain- Throbbing 80 osus is applied. Although migraine often Unilaterality* 61 begins in the morning, sometimes Moderate to 97 awakening the patient from sleep at severe* dawn, it can begin at any time of the Worse with 95 day or night. The frequency of attacks activity* is extremely variable, from a few in a lifetime to several in a week. The median Nausea* 87 68 attack frequency is 1.5 attacks per month; * Vomiting 56 10% of migraineurs have one or more Photophobia* 85 attacks per week (Stewart et al, 1994). * The diagnosis of migraine with aura Phonophobia 80 requires at least two attacks with any Allodynia 65 two of three features: (1) one or more Nasal 70 fully reversible aura symptoms; (2) the congestion aura developing over more than 4 min- Neck pain 65 utes but lasting less than 60 minutes; and (3) the headache following the Stabbing pain 40 aura with a free interval of less than 60 Clusterlike eye 5 minutes. Migraine with aura is subdi- symptoms vided into migraine with typical aura *In International Headache Society criteria. (homonymous visual disturbance, uni- lateral numbness, or aphasia); migraine Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited. KEY POINTS: A Most migraineurs TABLE 4-2 International Classification of Headache Disorders. 2nd Edition, with migraine Migraine Classification with aura also have attacks 1. Migraine without aura. A 1.1 Migraine without aura In contrast to a transient 1.2 Migraine with aura ischemic attack, 1.2.1 Typical aura with migraine headache the aura of migraine evolves 1.2.2 Typical aura with nonmigraine headache gradually and 1.2.3 Typical aura without headache typically consists 1.2.4 Familial hemiplegic migraine of both positive (eg, scintillations, 1.2.5 Sporadic hemiplegic migraine tingling) and 1.2.6 Basilar-type migraine negative (eg, scotoma, 1.3 Childhood periodic syndromes that are commonly numbness) precursors of migraine features. 1.3.1 Cyclical vomiting 1.3.2 Abdominal migraine 1.3.3 Benign paroxysmal vertigo of childhood 1.4 Retinal migraine 1.5 Complications of migraine 1.5.1 Chronic migraine 1.5.2 Status migrainosus 1.5.3 Persistent aura without infarction 1.5.4 Migrainous infarction 1.5.5 Migraine-triggered seizures 1.6 Probable migraine 1.6.1 Probable migraine without aura 1.6.2 Probable migraine with aura 69 1.6.5 Probable chronic migraine Data from Headache Classification Subcommittee. The International Classification of Headache Disorders. 2nd edition. Cephalalgia 2004;24(suppl 1):9–160. aura with nonmigraine headache or the headache, but occasionally it occurs without headache; familial and spo- with the headache or only during the radic hemiplegic migraine; and basilar headache. In contrast to a transient migraine. Other varieties of migraine ischemic attack (TIA), the aura of mi- include retinal migraine and childhood graine evolves gradually and typically periodic syndromes. Most patients with consists of both positive (eg, scintilla- migraine with aura also have attacks tions, tingling) and negative (eg, sco- without aura. The aura usually lasts 20 toma, numbness) features. If the aura to 30 minutes and typically precedes is stereotypical, the diagnosis of migraine Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited. " MIGRAINE: SYMPTOMS AND DIAGNOSIS KEY POINTS: with aura is warranted, even if the the headache. If the headache is ab- A Almost any subsequent headache does not have sent, it is migraine aura without head- symptom or sign of brain typical migrainous features. Almost ache. Both may be associated with dysfunction any symptom or sign of brain dysfunc- premonitory symptoms. (Premonitory may be a tion may be a feature of the aura, but symptoms often extend into the head- feature of the commonly the aura is visual. ache or may begin in the headache migraine aura, Focal symptoms and signs of the phase). but commonly aura may persist beyond the headache the aura is phase. Formerly termed complicated Premonitory Phase visual. migraine, the IHS classification has Premonitory phenomena occur hours A Particularly in introduced two more-specific labels. to days before headache onset in about mid or late life, If the aura lasts for more than 1 hour 60% of migraineurs and can consist of the migraine but less than 1 week, the term migraine psychological, neurological, or general aura may not with prolonged aura was formerly (constitutional, autonomic) symptoms be followed by applied, but now the episode is called (Table 4-4). Psychological symptoms