[Hallervorden-Spatz disease with Lewy bodies].
At the age of 6 years a patient developed disorders of character, intellectual deterioration, tremor, falls and epileptic seizures. This was followed by extrapyramidal and pyramidal disorders with a fatal outcome at age 21. There was no family history. Histopathology showed evidence of Hallervorden-Spatz disease, remarkable by the diffusion of spheroids into the central nervous system gray matter and by the presence of innumerable Lewy bodies in the substantia nigra and locus coeruleus. Similar findings have been reported in only 3 other cases of typical Hallervorden-Spatz disease. They suggest a preferential affection of monoaminergic neurons.
D000293:Adolescent; D000328:Adult; D001369:Axons / Q000473:pathology; D001479:Basal Ganglia / Q000473:pathology; D001480:Basal Ganglia Diseases / Q000473:pathology*; D001921:Brain / Q000473:pathology; D002648:Child; D003937:Diagnosis, Differential; D005917:Globus Pallidus / Q000473:pathology; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology* / Q000648:ultrastructure; D007501:Iron / Q000032:analysis; D008125:Locus Coeruleus / Q000473:pathology; D008297:Male; D009474:Neurons / Q000473:pathology* / Q000648:ultrastructure; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000175:diagnosis / Q000473:pathology*; D013378:Substantia Nigra / Q000473:pathology*
D007501:Iron
Maladie de Hallervorden-Spatz avec corps de Lewy.