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

页码：730 / 732

页数：3