Department of Neurology, Turku University Hospital, Kiinamyllynkatu 4-8, 20521, Turku, Finland.
J Headache Pain. 2012 Jan;13(1):95-7. doi: 10.1007/s10194-011-0400-y. Epub 2011 Nov 8.
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited disease of small arteries caused by mutations in the Notch3 gene. Complex migrainous episodes, such as acute confusional migraine, status migrainosus with persisting aura, and "CADASIL coma" have been described in patients with CADASIL. However, there are few descriptions of effective treatment of such episodes. We describe a 44-year-old male with CADASIL, who presented with sudden-onset aphasia and decreased responsiveness after prolonged, severe migraine attack. Subsequently, the patient had two generalized seizures. A subtle status epilepticus was suspected because of drowsiness and seizures, and intravenous sodium valproate medication was initiated. EEG recording showed left hemispheric attenuation but no spike discharges, thus not confirming epileptic mechanism. The clinical status of the patient improved markedly after the initiation of valproate. The patient started speaking again; drowsiness and headache subsided. In repeated EEG recording, the left hemispheric attenuation disappeared. Diffusion weighted MR imaging showed no signs of recent ischemic events. The patient recovered fully from the episode with no further seizures. We suggest that CADASIL patients with acute complex migrainous episodes may benefit from intravenous sodium valproate.
伴有皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病（CADASIL）是一种由 Notch3 基因突变引起的小动脉遗传性疾病。在 CADASIL 患者中，已经描述了复杂的偏头痛发作，如急性意识模糊性偏头痛、持续先兆的偏头痛状态和“CADASIL 昏迷”。然而，对于此类发作的有效治疗方法的描述却很少。我们描述了一位 44 岁的男性 CADASIL 患者，他在长时间严重偏头痛发作后出现突发性失语和反应迟钝。随后，患者出现了两次全身性癫痫发作。由于嗜睡和癫痫发作，怀疑存在轻微的癫痫持续状态，并开始静脉注射丙戊酸钠。脑电图记录显示左半球衰减，但没有尖峰放电，因此无法确认癫痫机制。丙戊酸钠开始使用后，患者的临床状况明显改善。患者再次开始说话；嗜睡和头痛减轻。在重复脑电图记录中，左半球衰减消失。弥散加权磁共振成像显示无近期缺血事件的迹象。患者完全从该发作中恢复，没有再发生癫痫。我们建议，伴有急性复杂偏头痛发作的 CADASIL 患者可能受益于静脉注射丙戊酸钠。
