Máttyus A, Veres E, Halász A
Psychiatr Neurol Med Psychol (Leipz). 1977 Feb;29(2):77-86.
Twenty-three cases of tuberous sclerosis, found in the ten years' material of the authors among 2900 epileptics between o-16 years of age, are presented. 11 patients were treated before the age of 3 years, all showing infantile spasms. The earliest, in most cases congenital extraneural signs were depigmented areas on the skin. They were absent only in one case. They allowed, together with the Westsyndrome to establish the diagnosis if tuberous sclerosis before the appearance of other characteristic symptoms. Sebaceous adenoma, generally accepted as pathognostic for tuberous sclerosis, presented itself in all 12 early cases later then the leukoderma and the epileptic fits. Tuberous sclerosis is one of the important causes of epilepsy in childhood, primarily in early childhood. In this period of life the West-syndrome is the most frequent type of epileptic manifestations of tuberous sclerosis
本文呈现了作者在10年研究材料中,于2900例0至16岁癫痫患者中发现的23例结节性硬化症病例。11例患者在3岁前接受治疗,均表现为婴儿痉挛症。在大多数情况下,最早出现的先天性神经外体征是皮肤上的色素脱失区域,仅1例未出现。在其他特征性症状出现之前,这些体征连同韦斯特综合征有助于确立结节性硬化症的诊断。皮脂腺瘤通常被认为是结节性硬化症的确诊依据,在所有12例早期病例中,其出现时间晚于白斑和癫痫发作。结节性硬化症是儿童癫痫的重要病因之一,主要发生在幼儿期。在这个生命阶段,韦斯特综合征是结节性硬化症最常见的癫痫表现类型