Vahedi Katayoun, Chabriat Hugues, Levy Claude, Joutel Anne, Tournier-Lasserve Elisabeth, Bousser Marie-Germaine
Service de Neurologie, Assistance Publique--Hôpitaux de Paris, Hôpital Lariboisière, Paris, France.
Arch Neurol. 2004 Aug;61(8):1237-40. doi: 10.1001/archneur.61.8.1237.
Migraine with aura (MA) is one of the clinical hallmarks of CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy), a small vessel disease of the brain caused by mutations in the NOTCH3 gene, but its exact mechanisms are unknown.
To describe the patterns of MA in CADASIL and to compare brain magnetic resonance signal abnormalities between CADASIL patients with and without MA.
Comparison of brain magnetic resonance signal abnormalities between cases and controls.
Patients with CADASIL seen at Lariboisière Hospital.
Forty-one CADASIL patients with MA and 31 age-matched CADASIL controls without MA.
The mean age at onset of MA was significantly younger in women compared with men and occurred a mean of 15 years prior to stroke onset. A majority of patients (56%) reported at least 1 migraine attack with atypical aura. All CADASIL patients either with or without MA had white matter signal abnormalities on T2-weighted imaging. There was no difference in the frequency and distribution of brain signal abnormalities between CADASIL patients with and without MA.
In CADASIL, MA is characterized by an unusually high frequency of attacks of migraine with atypical aura. The distribution and extent of magnetic resonance signal abnormalities did not differ according to migraine phenotype.
伴先兆偏头痛（MA）是大脑常染色体显性遗传性动脉病伴皮质下梗死和白质脑病（CADASIL）的临床特征之一，CADASIL是一种由NOTCH3基因突变引起的脑小血管疾病，但其确切机制尚不清楚。
描述CADASIL中MA的模式，并比较有和没有MA的CADASIL患者之间脑磁共振信号异常情况。
病例与对照之间脑磁共振信号异常情况的比较。
拉里博瓦西耶医院的CADASIL患者。
41例有MA的CADASIL患者和31例年龄匹配的无MA的CADASIL对照患者。
MA发病的平均年龄女性显著低于男性，且平均在卒中发作前15年出现。大多数患者（56%）报告至少有1次伴有非典型先兆的偏头痛发作。所有有或没有MA的CADASIL患者在T2加权成像上均有白质信号异常。有和没有MA的CADASIL患者之间脑信号异常的频率和分布没有差异。
在CADASIL中，MA的特征是伴有非典型先兆的偏头痛发作频率异常高。磁共振信号异常的分布和程度根据偏头痛表型并无差异。
