卡恩斯-塞尔综合征的非典型磁共振成像表现：T2加权像上的放射状条纹。

Atypical MRI findings in Kearns-Sayre syndrome: T2 radial stripes.

作者信息

Hourani R G, Barada W M, Al-Kutoubi A M, Hourani M H

机构信息

Department of Diagnostic Radiology, The American University of Beirut Medical Center, Riad El Solh 1107, 2020 Beirut, Lebanon.

出版信息

Neuropediatrics. 2006 Apr;37(2):110-3. doi: 10.1055/s-2006-924226.

DOI:10.1055/s-2006-924226

PMID:16773512

Abstract

Kearns-Sayre syndrome (KSS) is a mitochondrial disorder consisting of external ophthalmoplegia, retinitis pigmentosa, ataxia and heart block. Magnetic resonance imaging (MRI) shows abnormal T2 high signal intensity in the deep gray matter nuclei, the cerebellar and the subcortical white matter. We report an unusual MR pattern of KSS, where the T2 images revealed radially oriented, hypointense stripes in hyperintense white matter, a characteristic MRI pattern of lysosomal disease not previously reported in KSS.

摘要

卡恩斯-塞尔综合征（KSS）是一种线粒体疾病，由眼外肌麻痹、色素性视网膜炎、共济失调和心脏传导阻滞组成。磁共振成像（MRI）显示深部灰质核、小脑和皮质下白质T2高信号异常。我们报告了一例KSS不寻常的磁共振成像表现，T2图像显示在高信号白质中有放射状排列的低信号条纹，这是一种溶酶体疾病的特征性MRI表现，此前在KSS中未曾报道过。