Laubscher B, Deonna T, Uske A, van Melle G
Department of Paediatrics (Neuropaediatric Unit), Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
Eur J Pediatr. 1990 Apr;149(7):502-7. doi: 10.1007/BF01959405.
We studied 74 children with primitive megalencephaly retrospectively with attention directed to familial megalencephaly, birth history, enlarged pericerebral subarachnoid space (SAS) (idiopathic external hydrocephalus), head and statural growth dynamics, developmental and school prognosis, morphological findings and development of subdural haematoma. In the megalencephalic children, no significant differences were found between those with normal or those with enlarged pericerebral SAS. Out of 62, 31 children (50%) were already megalencephalic at birth. Of 74, 37 children (50%) showed variable degrees of developmental delay which in 18 was transient. Eight out of 74 were mentally retarded. Of 52 children at school age, 42 attend normal schools and 10, of whom 7 are mentally retarded, attend special schools. Three children showed subdural haematoma resulting from apparently minor trauma or occurring spontaneously. We suggest that idiopathic external hydrocephalus represents a variant of primitive megalencephaly with transient increase of intracranial pressure and that it could predispose to the development of idiopathic (spontaneous or non-traumatic) subdural haematoma.
我们对74例原发性巨脑症患儿进行了回顾性研究，重点关注家族性巨脑症、出生史、脑周蛛网膜下腔（SAS）增宽（特发性外部性脑积水）、头围和身高生长动态、发育及学业预后、形态学表现以及硬膜下血肿的发生情况。在巨脑症患儿中，脑周SAS正常者与增宽者之间未发现显著差异。62例患儿中，31例（50%）出生时即已出现巨脑症。74例患儿中，37例（50%）表现出不同程度的发育迟缓，其中18例为暂时性。74例中有8例智力发育迟缓。在52例学龄儿童中，42例就读于普通学校，10例就读于特殊学校，其中7例智力发育迟缓。3例患儿出现硬膜下血肿，原因明显为轻微外伤或自发形成。我们认为，特发性外部性脑积水是原发性巨脑症的一种变体，伴有颅内压短暂升高，且可能易引发特发性（自发或非创伤性）硬膜下血肿。
