D'Cruz O F, Vaughn B V, Gold S H, Greenwood R S
Department of Neurology, University of North Carolina-Chapel Hill, 27599.
Neurology. 1994 Nov;44(11):2189-91. doi: 10.1212/wnl.44.11.2189.
Cataplexy is a cardinal manifestation of the narcolepsy syndrome. Although symptomatic narcolepsy is well described, isolated cataplexy is extremely rare. We reviewed clinical and radiologic data in two patients with isolated symptomatic cataplexy and associated CNS disease. In an HLA-DR2-positive patient with chronic progressive MS, we confirmed cataplexy by observation of reported spells. MRI revealed diffuse white-matter lesions involving the medial medulla, pons, and subcortical white matter; protriptyline provided symptomatic relief. A second patient with a pontomedullary pilocytic astrocytoma developed infrequent but recurrent cataplectic attacks in association with sleep fragmentation due to nocturnal cough and nausea. MRI revealed an enhancing lesion involving the dorsal pons and medulla. Genetic predisposition and sleep fragmentation may precipitate symptomatic cataplexy in patients with structural pontomedullary lesions.
猝倒症是发作性睡病综合征的主要表现。尽管有症状的发作性睡病已有详尽描述，但孤立性猝倒症极为罕见。我们回顾了两名患有孤立性症状性猝倒症及相关中枢神经系统疾病患者的临床和影像学资料。在一名患有慢性进行性多发性硬化症的 HLA - DR2 阳性患者中，通过观察其报告的发作情况确诊为猝倒症。磁共振成像（MRI）显示弥漫性白质病变累及延髓内侧、脑桥和皮质下白质；普罗替林提供了症状缓解。第二名患有桥延髓毛细胞型星形细胞瘤的患者，因夜间咳嗽和恶心导致睡眠片段化，出现了不频繁但反复发作的猝倒发作。MRI 显示一个强化病变累及脑桥背侧和延髓。遗传易感性和睡眠片段化可能促使患有桥延髓结构性病变的患者出现症状性猝倒症。
