Late onset parkinsonian syndrome in Hallervorden-Spatz disease.
Two siblings, from consanguineous parents, developed in their twenties a Parkinsonian syndrome. In the elder, the disease evolved for 13 years and the necropsic study was diagnostic of Hallervorden-Spatz disease. The younger sibling is severely affected after 12 years of the disorder. Several CT and one MR studies done in this patient during the last 4 years have been normal. Ultrastructural studies of the bone marrow histiocytes and blood lymphocytes disclosed peculiar inclusions. Bromocriptine in low doses proved to be a beneficial therapy for this patient.
Alberca
R
R
Department of Neurology, Ciudad Sanitaria Virgen del Rocĩo, Sevilla, Spain.
Rafel
E
E
Chinchon
I
I
Vadillo
J
J
Navarro
A
A
D000328:Adult; D001480:Basal Ganglia Diseases / Q000503:physiopathology*; D001921:Brain / Q000473:pathology* / Q000648:ultrastructure; D003241:Consanguinity; D005260:Female; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*; D010302:Parkinson Disease, Secondary / Q000209:etiology* / Q000473:pathology