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伴有双侧颞叶囊肿的非进行性白质脑病

Non-progressive leukoencephalopathy with bilateral temporal cysts.

作者信息

Gomes A L, Vieira J P, Saldanha J

机构信息

Department of Pediatrics, Hospital de Santa Maria, Lisboa, Portugal.

出版信息

Eur J Paediatr Neurol. 2001;5(3):121-5. doi: 10.1053/ejpn.2001.0482.

Abstract

We report two cases of a peculiar leukoencephalopathy with temporal cysts. Both patients have a non-progressive neurological disorder with mental retardation, microcephaly and sensorineural deafness although clinical differences between them may reflect a different aetiology. The metabolic disorders with white matter involvement and the recently described leukoencephalopathies (Van Der Knaap disease, 'vanishing white matter disease') were excluded based on clinical, biologic and imaging findings. Cytomegalovirus infection is a likely possibility in the first case although the magnetic resonance imaging picture is only partially similar to previously reported cases. Our patients are strikingly similar to the patients reported by Deonna et al. and Olivier et al. We discuss the clinical and imaging findings in our patients and the differential diagnosis considering the known disorders of the white matter in childhood.

摘要

我们报告了两例伴有颞叶囊肿的特殊白质脑病病例。两名患者均患有非进行性神经系统疾病,伴有智力发育迟缓、小头畸形和感音神经性耳聋,尽管他们之间的临床差异可能反映了不同的病因。根据临床、生物学和影像学检查结果,排除了伴有白质受累的代谢性疾病以及最近描述的白质脑病(范德克纳普病、“消失性白质病”)。巨细胞病毒感染在第一例中很有可能,尽管磁共振成像表现仅部分类似于先前报道的病例。我们的患者与德奥纳等人和奥利维耶等人报告的患者极为相似。我们讨论了我们患者的临床和影像学检查结果以及考虑到儿童期已知白质疾病的鉴别诊断。

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