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Migraine with aura is characterized by transient focal neurological symptoms called aura. Visual, sensory, and dysphasic aura are the most common symptoms, but other aura variants such as motor aura may occur. The visual and sensory aura are generally characterized by positive symptoms (flickering light, zigzag lines, or prickling paresthesias) followed by negative symptoms (blind spot and numbness). Furthermore, the aura symptoms usually develop gradually, over a few minutes, and often spread over the area of initial clinical manifestation (across the visual field, from one hand to the lower arm, or across the face). The aura duration is usually less than one hour. The aura symptoms may be followed by headache, nausea, vomiting, or photo-or phonophobia as seen in migraine without aura, but aura attacks may also occur in isolation. This chapter describes the classification and clinical features of migraine with aura.

Objective.—To compare the clinical characteristics of familial hemiplegic migraine (FHM), sporadic hemiplegic migraine (SHM), and nonhemiplegic migraine with aura (NHMA) and further, to compare subtypes of NHMA. Background.—To discover distinct underlying genetic and pathophysiological mechanisms it is crucial to drive clinical subdivision of migraine with aura as far as possible. The documentation of subtypes of migraine with aura depends on the clinical characteristics as the genetic mechanisms are unknown except for the dominantly inherited FHM. Methods.—Patients with FHM, SHM, or familial NHMA were recruited from specialist practice and diagnosed according to the International Classification of Headache Disorders (ICHD) in a validated interview by a physician. Patients with hemiplegic migraine had a physical and neurological examination. Patients with population-based NHMA from a previous Danish study were used for comparison. Results.—We recruited 147 patients with FHM, 105 with SHM, and 362 with familial NHMA. FHM and SHM had similar aura and headache characteristics. Patients with FHM and SHM were more likely to experience two or more aura symptoms (100% vs. 31%, P < .0001), they more often had prolonged aura symptoms, they almost always had a headache associated with the aura (93% vs. 58%, P < .0001), and they more frequently had basilar-type symptoms (69% vs. 10%, P < .0001) than patients with population-based NHMA. Patients with familial NHMA were more likely to experience two or more aura symptoms than patients with population-based NHMA (61% vs. 32%, P < .0001). Within the subtypes of NHMA, patients with typical aura with migraine headache had an earlier age at onset (20 ± 10 vs. 23 ± 13 years, P= .044) and a higher co-occurrence of migraine without aura (43% vs. 22%, P= .002) than patients with typical aura with nonmigraine headache. Conclusions.—The present study proves that distinct subtypes of migraine with aura exist. It further underlines the phenotypic differences between the different subtypes of migraine with aura which likely are caused by different etiological mechanisms. In future studies FHM, SHM, and NHMA therefore should be analyzed as separate entities and patients with NHMA may be stratified by ICHD-2 subtype of NHMA.

The International Classification of Headache Disorders 2nd edition (ICHD-2) subdivides migraine with aura (MA) differently from the ICHD-1 and includes new diagnostic criteria. The aim of the present study was to evaluate how the new classification works in practice and in comparison with the ICHD-1. The patients were recruited from a screen of the Danish National Patient Registry and from Danish neurologists. We included 362 patients diagnosed with MA according to the ICHD-1 in a validated semistructured physician-conducted interview. According to the ICHD-2, 89% (322 of 362) had MA and 11% (40 of 362) had probable MA. The MA patients had one or more ICHD-2 subtype of MA: 54% (173 of 322) had typical aura with migraine headache (MA-MH), 40% (129 of 322) had typical aura with non-migraine headache (MA-NMH), 37% (120 of 322) had aura without headache (MA-WOH), and 7% (26 of 322) had basilar-type migraine (MA-B). Of patients with MA-MH 34% (59 of 173) had co-occurrence of MA-WOH, 9% (16 of 173) had co-occurrence of MA-B and 5% (8 of 173) had co-occurrence of both MA-WOH and MA-B. Of patients with MA-NMH 27% (35 of 129) had co-occurrence of MA-WOH. Only 6% (18 of 322) of the MA patients had exclusively MA-WOH and

The objectives of the present study were to describe the clinical characteristics of patients with severe familial non-hemiplegic migraine with aura (NHMA) and to compare these data to those from cases in previous population-based Danish studies using the same methodology. NHMA families were recruited from the Danish patient registry and from Danish neurology practices. A total of 362 NHMA patients were diagnosed according to the 1988 International Headache Society criteria using a validated semistructured physician-conducted interview. Visual aura occurred in almost every NHMA attack. In aura without headache visual aura occurred primarily in isolation. Aura without headache was most common in older, male patients. Several clinical characteristics of familial NHMA differed from migraine with aura in the general population: firstly, the age at onset was lower, secondly, the age at cessation was higher, thirdly, aura symptoms were more severe and finally, the co-occurrence of migraine without aura was higher in familial NHMA. There seems to be a correlation between more severe symptoms and familial aggregation. These results have both clinical and scientific implications.

Familial hemiplegic migraine (FHM) is a rare autosomal dominantly inherited subtype of migraine with aura. The clinical characteristics of FHM have been described previously in selected materials or case studies, but population-based studies are important in order to analyse the full spectrum of the disorder. The aim of the present study was to perform a systematic search for familial cases of migraine with an aura that included motor weakness in order to generate non-selected material of as many FHM cases as possible in the Danish population of 5.2 million inhabitants, and to compare this material with already available population-based clinical descriptions of migraine with typical aura (MA). Due to the rarity of FHM, traditional population-based methods were not feasible. Therefore, the search strategy employed a computer search of the National Patient Register, screening >27 000 case records from headache clinics and private neurologists, and advertisements. A total of 147 affected FHM patients from 44 families were identified. FHM patients most often had all four 'typical' aura symptoms (visual, sensory, aphasic and motor symptoms) and all had at least two of these aura symptoms during FHM attacks. The motor, sensory and visual aura symptoms were all similar in type to the motor, sensory and visual aura symptoms in MA, but FHM had a statistically significantly longer duration of the visual and sensory aura symptoms, and these and other aura symptoms often fulfilled the criteria of the International Headache Society for prolonged aura. In addition, 69% had basilar migraine (BM) symptoms during FHM attacks. The order of the aura symptoms was usually visual, followed by sensory, aphasic, motor and, lastly, basilar-type migraine symptoms. Headache was present in 99% of FHM patients during FHM attacks, whereas the aura symptoms more often occurred without headache in MA. Headache duration was significantly longer in FHM compared with MA. Based on these data, we suggest more precise diagnostic criteria for FHM and a more clear clinical distinction between FHM and BM. Our results have significant implications for case finding in genetic studies and for clinical migraine differential diagnosis.

To supplement the traditional ICHD-2 diagnosis for migraine with aura (MA) we developed a diagnostic scale for migraine aura that quantifies the importance of the cardinal characteristics of MA. Since more than 99% of MA patients have visual aura, we developed for simplicity a Visual Aura Rating Scale (VARS). In total 427 patients with MA (ICHD-2) or nonaura visual disturbances were diagnosed in a validated semistructured interview by a trained physician. The patients were separated into a derivation sample and a validation sample. By regression analysis we identified the visual aura characteristics associated with MA in the derivation sample. Based on the identified characteristics we developed VARS and derived a predictive VARS score which was tested in the validation sample. The VARS score is the weighted sum of the presence of five visual symptom characteristics: duration 5-60 min (3 points), develops gradually ≤5 min (2 points), scotoma (2 points), zig-zag lines (2 points), and unilateral (1 point). The maximum score is 10 points. A VARS score of 5 or more diagnosed MA with a sensitivity of 96% (95% CI 92-99%) and a specificity of 98%(95% CI 95-100%) in the derivation sample, and a sensitivity of 91% (95% CI 86-95%) and a specificity of 96% (95% CI 91-100%) in the validation sample. VARS adds evidence based weights to a number of clearly specified characteristics; it is easy to learn, apply and teach and may therefore be a valuable addition to traditional ICHD-2 diagnosis.

The present study is a 16-year follow-up study assessing the long-term outcome of migraine with aura (MA). Additionally possible predictive factors in the prognosis of MA were evaluated. Patients were recruited from the files of Danish headache clinics. A total of 53 patients (11M:42F) with MA (IHS criteria) participated in a follow-up interview. At follow-up attacks had ceased (no MA for 2 years) in 36% of patients. Attacks had ceased in 55% of males and 31% of females ( P = 0.17). Attacks had ceased in 41% of patients with visual aura without other aura symptoms and in 25% of those with sensory or aphasic aura besides their visual aura ( P = 0.36). Among those with attacks of MA at follow-up frequency of attacks and headache intensity was improved in 44% and 41% of the patients, respectively. The results point to a favourable evolution of MA and suggest possible predictive factors.