title: [Hallervorden Spatz syndrome: magnetic resonance findings. Case report].
abstract: 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.
authors.affiliation: Hospital de Clínicas (HC), Universidade Federal de Uberlândia, Uberlândia, MG, Brazil. lucianofarage@yahoo.com.br
authors.forename: Luciano
authors.initials: L
authors.lastname: Farage
authors.forename: Mário Augusto Padula
authors.initials: MA
authors.lastname: Castro
authors.forename: Túlio Augusto Alves
authors.initials: TA
authors.lastname: Macedo
authors.forename: Marcelo Cardoso de
authors.initials: MC
authors.lastname: Assis
authors.forename: Lincoln Pereira de
authors.initials: LP
authors.lastname: Souza
authors.forename: Luiz Oliveira de
authors.initials: LO
authors.lastname: Freitas
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007501:Iron / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000175:diagnosis* / Q000209:etiology
chemical_list: D007501:Iron