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Ann Neurosci. 2011 Jul;18(3):133-5. doi: 10.5214/ans.0972.7531.11183012.

Hallervorden-Spatz syndrome: a rare cause of extrapyramidal manifestations.

Annals of neurosciences

Kv Vinod, S Giridharan, Tk Dutta

Affiliations

  1. Department of General Medicine, JIPMER, Dhanvantarinagar, Puducherry- 605 006, INDIA.

PMID: 25205940 PMCID: PMC4116949 DOI: 10.5214/ans.0972.7531.11183012

Abstract

Hallervorden-Spatz syndrome is a rare neurodegenerative disease of autosomal recessive inheritance which presents in childhood or early adulthood with dystonia, dysarthria, rigidity and choreoathetosis. Here we present an unusual case of atypical Hallervorden-Spatz syndrome with onset during adolescence and rapid progression in a young female patient who showed the characteristic "eye of the tiger" appearance on magnetic resonance imaging [MRI] of brain. This reporting intends to highlight Hallervorden-Spatz syndrome as a rare cause of extrapyramidal manifestations and the interesting radiologic picture of the disease.

Keywords: dystonia; extrapyramidal; eye of the tiger; neurodegeneration; pantothenate kinase 2

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