[Hallervorden Spatz syndrome: magnetic resonance findings. Case report]

Autor: Luciano, Farage, Mário Augusto Padula, Castro, Túlio Augusto Alves, Macedo, Marcelo Cardoso de, Assis, Lincoln Pereira de, Souza, Luiz Oliveira de, Freitas
Rok vydání: 2004
Předmět:
Zdroj: Arquivos de neuro-psiquiatria. 62(3A)
ISSN: 0004-282X
Popis: 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.
Databáze: OpenAIRE