"Hallervorden-Spatz syndrome--infantile neuroaxonal dystrophy" complex. Case report

Case report of a 7 1/2-year-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 putamen,...

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Bibliographic Details
Published in:Arquivos de neuro-psiquiatria 1988-03, Vol.46 (1), p.69-72
Main Authors: Bresolin, A U, Pascuzzi, L, Melaragno Filho, R, Fontana, M H, Pecora, R, Souza Dias, J C
Format: Article
Language:English
Subjects:
Axons - ultrastructure
Basal Ganglia Diseases - pathology
Child
Female
Humans
NEUROSCIENCES
Pantothenate Kinase-Associated Neurodegeneration - pathology
Pantothenate Kinase-Associated Neurodegeneration - physiopathology
PSYCHIATRY
Tomography, X-Ray Computed
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Summary:Case report of a 7 1/2-year-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 putamen, with no signs of cerebral atrophy. Pathological examination disclosed an intense red coloration of the putamen and axonal "spheroids" at electron microscopy.
ISSN:0004-282X
1678-4227
0004-282X
DOI:10.1590/s0004-282x1988000100012