'Hallervorden-Spatz syndrome - infantile neuroaxonal dystrophy' complex: case report

Case report of a 7 1/2-years old girl considered as being normal until the age of 2 years. From then on she progressed with gait disturbance, mental deterioration, dystonic movements, convulsions and dysarthria. She died of bronchopneumonia one year later. CT scan showed hyperdensity at the putamina...

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Bibliographic Details
Main Authors: A. U. Bresolin, L. Pascuzzi, R. Melaragno Filho, Maria H. Fontana, R. Pécora, J. C. Souza Dias
Format: Article
Language:English
Published: Thieme Revinter Publicações 1988-03-01
Series:Arquivos de Neuro-Psiquiatria
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X1988000100012&lng=en&tlng=en
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Summary:Case report of a 7 1/2-years old girl considered as being normal until the age of 2 years. From then on she progressed with gait disturbance, mental deterioration, dystonic movements, convulsions and dysarthria. She died of bronchopneumonia one year later. CT scan showed hyperdensity at the putamina, with no signs of cerebral atrophy. Pathological examination disclosed an intense red coloration of the putamina and axonal «spheroids» at electron microscopy.
ISSN:1678-4227