选定的脑白质营养不良症。

Selected leukodystrophies.

作者信息

Powers J M, Rubio A

机构信息

Department of Pathology and Laboratory Medicine, University of Rochester Medicine Center, NY 14642, USA.

出版信息

Semin Pediatr Neurol. 1995 Sep;2(3):200-10. doi: 10.1016/s1071-9091(05)80031-2.

DOI:10.1016/s1071-9091(05)80031-2

PMID:9422247

Abstract

The clinicopathologic profiles of the major leukodystrophies (adreno-leukodystrophy, metachromatic leukodystrophy, globoid cell leukodystrophy or Krabbe's disease, Pelizaeus-Merzbacher disease, and spongy degeneration of infancy or Canavan's disease) are reviewed. Particular attention is paid to distinctive imaging characteristics, molecular advances, pathogeneses, and potential therapies.

摘要

本文回顾了主要脑白质营养不良症（肾上腺脑白质营养不良、异染性脑白质营养不良、球形细胞脑白质营养不良或克拉伯病、佩利措伊斯-梅茨巴赫病以及婴儿海绵状变性或卡纳万病）的临床病理特征。特别关注了其独特的影像学特征、分子进展、发病机制和潜在治疗方法。