title: Generalized freezing in Hallervorden-Spatz syndrome: case report.
abstract: We report the case of a female patient who was exhibiting slowly progressive, severe, generalized freezing of voluntary movement and disequilibrium. Brain magnetic resonance imaging showed a very low signal intensity in both pallidal nuclei, with a high signal intensity in the central portion, the so called 'eye-of-the-tiger' sign. Despite the unusual clinical features, we believe that this is a case of Hallervorden-Spatz syndrome, although without neuropathological examination we were unable to confirm the diagnosis with certainty. This case further demonstrates the complex role of the pallidum in voluntary movement.
authors.affiliation: Servico de Neurologia, Hospital de Egas Moniz - Rua da Junqueira, 126-1300, Lisboa, Portugal.
authors.forename: J
authors.initials: J
authors.lastname: Guimarães
authors.forename: J V
authors.initials: JV
authors.lastname: Santos
mesh_terms: D000328:Adult; D001519:Behavior; D001921:Brain / Q000473:pathology; D018450:Disease Progression; D004576:Electromyography; D005260:Female; D006236:Handwriting; D006801:Humans; D008279:Magnetic Resonance Imaging; D009069:Movement Disorders / Q000473:pathology / Q000503:physiopathology*; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000473:pathology / Q000503:physiopathology*; D013060:Speech