[Hallervorden Spatz syndrome: magnetic resonance findings. Case report].
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.
Farage
Luciano
L
Hospital de Clínicas (HC), Universidade Federal de Uberlândia, Uberlândia, MG, Brazil. lucianofarage@yahoo.com.br
Castro
Mário Augusto Padula
MA
Macedo
Túlio Augusto Alves
TA
Assis
Marcelo Cardoso de
MC
Souza
Lincoln Pereira de
LP
Freitas
Luiz Oliveira de
LO
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
D007501:Iron