"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, with no signs of cerebral atrophy. Pathological examination disclosed an intense red coloration of the putamen and axonal "spheroids" at electron microscopy.
Bresolin
A U
AU
Servico de Neurologia, Hospital do Servidor Público Estadual de São Paulo, Brasil.
Pascuzzi
L
L
Melaragno Filho
R
R
Fontana
M H
MH
Pecora
R
R
Souza Dias
J C
JC
D001369:Axons / Q000648:ultrastructure*; D001480:Basal Ganglia Diseases / Q000473:pathology*; D002648:Child; D005260:Female; D006801:Humans; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000473:pathology* / Q000503:physiopathology; D014057:Tomography, X-Ray Computed