Camfield Peter, Camfield Carol, Lortie Anne, Darwish Husam
Dalhousie University and the IWK Grace Health Centre, PO Box 3070, Halifax, Nova Scotia, Canada B3J 3G9.
Epilepsia. 2003 Dec;44(12):1592-5. doi: 10.1111/j.0013-9580.2003.29203.x.
West syndrome consists of infantile spasms with hypsarrhythmia and is perceived as a disorder of infants.
We describe 10 patients with West syndrome with spasms that remitted, started again, and persisted (followed up for 8-25 years).
In all, West syndrome developed at younger than 17 months (five cryptogenic, six symptomatic). With initial treatment, spasms completely stopped for 4.5 months to 6 years, when epileptic spasms returned. Recurrent spasms were typical with brief arm extension, eye elevation, and head drop without falling. Spasms lasted 2-6 s in rhythmic strings over 20- to 60-min periods and occurred daily throughout follow-up. Persistent spasms were particularly troublesome, because of incontinence in one and postictal confusion in several. During the string of spasms, most refused to interact, and several would wander off. Up to 15 antiepileptic drugs did not render any patient spasm free. Only two had persistent spasms as the only seizure type; six also had intractable complex partial seizures, and three had occasional grand mal convulsions. Interictal EEGs showed multifocal spikes. Ictal recordings in six showed electrodecremental events.
Recurrent spasms after remission of West syndrome represent an extremely resistant, distressing form of epilepsy. The onset of West syndrome is age related, but it does not reliably vanish.
韦斯特综合征由婴儿痉挛伴高度失律组成，被认为是一种婴儿期疾病。
我们描述了10例韦斯特综合征患者，其痉挛缓解后又复发并持续存在（随访8 - 25年）。
总体而言，韦斯特综合征在17个月之前发病（5例隐源性，6例症状性）。经过初始治疗，痉挛完全停止4.5个月至6年，之后癫痫性痉挛复发。复发的痉挛表现为典型的短暂手臂伸展、眼球上抬和头部下垂但不跌倒。痉挛以有节律的串发形式持续2 - 6秒，发作周期为20至60分钟，在整个随访期间每天发作。持续性痉挛尤其麻烦，因为有1例出现尿失禁，几例出现发作后意识模糊。在一连串痉挛发作期间，大多数患者拒绝互动，有几例还会走失。多达15种抗癫痫药物都未能使任何患者完全无痉挛发作。只有2例仅以持续性痉挛作为唯一发作类型；6例还伴有难治性复杂部分性发作，3例偶尔出现大发作惊厥。发作间期脑电图显示多灶性棘波。6例患者的发作期记录显示有电极衰减事件。
韦斯特综合征缓解后复发的痉挛代表一种极具耐药性、令人痛苦的癫痫形式。韦斯特综合征的发病与年龄有关，但并非一定会消失。
