Sturm J W, Andermann F, Berkovic S F
Department of Neurology, University of Melbourne, Austin and Repatriation Medical Centre, Heidelberg, Victoria, Australia.
Neurology. 2000 Feb 22;54(4):971-3. doi: 10.1212/wnl.54.4.971.
Gelastic seizures are the hallmark of the epilepsy syndrome associated with hypothalamic hamartomas. Patients typically develop cognitive deterioration and refractory seizures. The authors describe three patients with small hypothalamic hamartomas without these features and thus identify a mild end to the clinical spectrum. All had the unusual symptom of "pressure to laugh," often without actual laughter. This symptom could be dismissed as psychogenic but should be recognized as a clue to the presence of this unusual lesion.
痴笑性癫痫是与下丘脑错构瘤相关的癫痫综合征的标志。患者通常会出现认知功能衰退和难治性癫痫发作。作者描述了三名患有小型下丘脑错构瘤但无这些特征的患者,从而确定了该临床谱系的一种轻度表现形式。所有患者都有“想笑的冲动”这一不寻常症状,通常并无实际发笑。这种症状可能被认为是心因性的,但应被视为存在这种不寻常病变的一个线索。