Hallervorden Spatz syndrome: magnetic resonance findings. Case report

被引：2
|
作者：
Farage, L [1 ]
Castro, MAP [1 ]
Macedo, TAA [1 ]
de Assis, MC [1 ]
de Souza, LP [1 ]
de Freitas, LO [1 ]
机构：
[1] Univ Fed Uberlandia, Hosp Clin, Setor Radiol, BR-38400 Uberlandia, MG, Brazil
来源：
ARQUIVOS DE NEURO-PSIQUIATRIA | 2004年 / 62卷 / 3A期
关键词：
Hallervorden Spatz syndrome; magnetic resonance imaging; eye-of-the-tiger sign;
D O I：
10.1590/S0004-282X2004000400031
中图分类号：
Q189 [神经科学];
学科分类号：
071006 ;
摘要：
Hallervorden-Spatz syndrome is a neurodegenerative disease, autosomic recessive with two clinical features: early and late onset. Psychiatric, pyramidal and extrapyramidal signs are present in the late subtype. We report the case of a 41-old woman with extrapyramidal signs. Magnetic resonance imaging (MRI) showed the eye-of-the-tiger sign in the medial globus pallidus. This is due to a gliosis (increased signal) and accumulation of surrounding iron (decreased signal intensity) in long TR sequences. There is a strong relationship between MRI findings and the gene mutation responsable for this disease. It makes the MRI sensible for diagnosing this syndrome.
引用
收藏
页码：730 / 732
页数：3
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