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Hallervorden Spatz syndrome: magnetic resonance findings. Case report

Hallervorden Spatz syndrome: magnetic resonance findings. Case report

Arquivos de Neuro-Psiquiatria 62(3a): 730-732

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.

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Accession: 049185889

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PMID: 15334240

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