磁共振成像显示典型前脑无裂畸形中白质成熟异常。

MRI shows abnormal white matter maturation in classical holoprosencephaly.

作者信息

Barkovich A J, Simon E M, Glenn O A, Clegg N J, Kinsman S L, Delgado M, Hahn J S

机构信息

Department of Diagnostic Radiology, University of California, San Francisco 94143, USA.

出版信息

Neurology. 2002 Dec 24;59(12):1968-71. doi: 10.1212/01.wnl.0000038354.10891.0e.

DOI:10.1212/01.wnl.0000038354.10891.0e

PMID:12499493

Abstract

In an attempt to assess white matter maturation in holoprosencephaly (HPE), MRI scans of 47 patients with HPE were retrospectively reviewed. White matter maturation was delayed in 25/47 patients, including 24/29 patients with classic HPE who were </=12 months old at the time of imaging. Of interest, all six patients with the middle interhemispheric (MIH) variant of HPE had normal myelination, regardless of age. This study provides further evidence that the classic and MIH variants of HPE have different underlying causes.

摘要

为了评估全前脑畸形（HPE）中的白质成熟情况，我们回顾性地分析了47例HPE患者的MRI扫描结果。47例患者中有25例白质成熟延迟，其中包括29例经典型HPE患者中的24例，这些患者在成像时年龄≤12个月。有趣的是，所有6例中间脑间（MIH）型HPE患者的髓鞘形成均正常，与年龄无关。这项研究进一步证明，HPE的经典型和MIH型有不同的潜在病因。