Stachniak J B, Mickle J P, Ellis T, Quisling R, Rojiani A M
Department of Neurosurgery, University of Florida College of Medicine, Gainsville 32610, USA.
Pediatr Neurosurg. 1995;22(5):266-9. doi: 10.1159/000120912.
A rare case of myelinoclastic diffuse sclerosis (MDS), occasionally referred to as Schilder's disease, is reported in a child with Turner's syndrome. The child originally presented with a 3-week history of nausea, vomiting and frontal headaches. Magnetic resonance imaging showed a large, contrast-enhancing, right frontal lobe mass which was ultimately resected uneventfully. Complete laboratory investigations and pathological evaluation of the resected specimen verified the case to be MDS. The clinical presentation, laboratory evaluation, imaging characteristics and diagnosis are discussed in this review of the disease. The importance of including demyelinating diseases in the differential diagnosis for newly discovered mass lesions in the pediatric population is underscored by this case.
本文报道了一例罕见的脱髓鞘性弥漫性硬化症（MDS），该病偶尔也被称为席尔德病，患者为一名患有特纳综合征的儿童。该患儿最初出现了为期3周的恶心、呕吐和前额头痛症状。磁共振成像显示右侧额叶有一个大的、有强化的肿块，最终该肿块被顺利切除。对切除标本进行的全面实验室检查和病理评估证实该病例为MDS。本文在对该疾病的综述中讨论了其临床表现、实验室评估、影像学特征及诊断。该病例强调了在儿科人群新发现的肿块病变鉴别诊断中纳入脱髓鞘疾病的重要性。
