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Generalized freezing in Hallervorden-Spatz syndrome: case report
1Servico de Neurologia, Hospital de Egas Moniz - Rua da Junqueira, 126-1300, Lisboa, Portugal.
European Journal of Neurology
|June 11, 1999
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.