Thalamic lesions in a long-surviving child with spinal muscular atrophy type I: MRI and EEG findings

被引：29

作者：

Ito, Y

Kumada, S

Uchiyama, A

Saito, K

Osawa, M

Yagishita, A

Kurata, K

Hayashi, M

机构：

[1] Tokyo Womens Med Univ, Sch Med, Dept Pediat, Shinjuku Ku, Tokyo 1628666, Japan

[2] Metropolitan Fuchu Med Ctr Severe Motor & Intelle, Dept Pediat, Tokyo, Japan

[3] Tokyo Metropolitan Neurol Hosp, Dept Neuroradiol, Tokyo, Japan

[4] Tokyo Metropolitan Inst Neurosci, Dept Clin Neuropathol, Tokyo, Japan

来源：

BRAIN & DEVELOPMENT | 2004年 / 26卷 / 01期

关键词：

spinal muscular atrophy (SMA) type I; magnetic resonance imaging (MRI); electroencephalography (EEG); thalamic lesions;

D O I：

10.1016/S0387-7604(03)00075-5

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Brain magnetic resonance imaging was conducted in a girl with genetically confirmed spinal muscular atrophy (SMA) type I. This patient has survived 6 years, to date, under mechanical ventilation. T2-weighted and fluid-attenuated inversion recovery images revealed high signal intensity lesions in the anterolateral portions of the bilateral thalami. Electroencephalography disclosed diffuse beta activity upon awakening and during light sleep. In addition, fast and prolonged spindles were observed. Although mild neuronal changes in the lateral nucleus of the thalamus have been described in several autopsied cases, this is the first study to demonstrate neuroradiologically and neurophysiologically the thalamic lesions in genetically confirmed SMA type I. (C) 2003 Elsevier B.V. All rights reserved.

引用

页码：53 / 56

页数：4

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