Head and Neck Research Group, Research Centre, Akershus University Hospital, Lørenskog, Oslo, Norway.
Lancet Neurol. 2011 May;10(5):457-70. doi: 10.1016/S1474-4422(11)70048-5. Epub 2011 Mar 30.
Hemiplegic migraine is a rare form of migraine with aura that involves motor aura (weakness). This type of migraine can occur as a sporadic or a familial disorder. Familial forms of hemiplegic migraine are dominantly inherited. Data from genetic studies have implicated mutations in genes that encode proteins involved in ion transportation. However, at least a quarter of the large families affected and most sporadic cases do not have a mutation in the three genes known to be implicated in this disorder, suggesting that other genes are still to be identified. Results from functional studies indicate that neuronal hyperexcitability has a pivotal role in the pathogenesis of hemiplegic migraine. The clinical manifestations of hemiplegic migraine range from attacks with short-duration hemiparesis to severe forms with recurrent coma and prolonged hemiparesis, permanent cerebellar ataxia, epilepsy, transient blindness, or mental retardation. Diagnosis relies on a careful patient history and exclusion of potential causes of symptomatic attacks. The principles of management are similar to those for common varieties of migraine, except that vasoconstrictors, including triptans, are historically contraindicated but are often used off-label to stop the headache, and prophylactic treatment can include lamotrigine and acetazolamide.
偏瘫性偏头痛是一种罕见的伴有先兆的偏头痛，涉及运动性先兆（无力）。这种类型的偏头痛可以是散发性的，也可以是家族性的。家族性偏瘫性偏头痛呈显性遗传。遗传研究的数据表明，编码参与离子转运的蛋白的基因突变与这种疾病有关。然而，至少四分之一受影响的大家族和大多数散发性病例在三个已知与这种疾病有关的基因中没有突变，这表明还有其他基因有待确定。功能研究的结果表明，神经元过度兴奋在偏瘫性偏头痛的发病机制中起着关键作用。偏瘫性偏头痛的临床表现从短暂性偏瘫发作到严重形式的反复昏迷和长时间偏瘫、永久性小脑共济失调、癫痫、短暂性失明或智力迟钝不等。诊断依赖于仔细的病史和排除症状性发作的潜在原因。治疗原则与常见偏头痛相同，只是血管收缩剂，包括曲坦类药物，历史上是禁忌的，但通常被非适应证用于停止头痛，预防治疗可以包括拉莫三嗪和乙酰唑胺。
