Neuroaxonal dystrophy with neuromelanin deposition, neurofibrillary tangles, and neuronal loss. Light- and electron-microscopic changes in a 45-year-old woman with progressive psychomotor deterioration.
Neuroaxonal spheroids became evident microscopically after the autopsy of a 45-year-old woman with pigmentation of the globus pallidus suggesting Hallervorden-Spatz disease. In our opinion the fine floccular pigment seen electron-microscopically in many of the axonal spheroids is melanin, an end product of catecholamine metabolism. Neurofibrillary degeneration, senile plaques, and granulovacuolar degeneration in the hippocampus produced a picture of Alzheimer's disease. Pontocerebellar degeneration and motor neuron disease were also observed.
D001369:Axons / Q000648:ultrastructure; D001927:Brain Diseases / Q000473:pathology*; D002531:Cerebellum / Q000648:ultrastructure; D005260:Female; D005917:Globus Pallidus / Q000648:ultrastructure; D006624:Hippocampus / Q000648:ultrastructure; D006801:Humans; D008543:Melanins / Q000032:analysis; D008854:Microscopy, Electron; D008875:Middle Aged; D009046:Motor Neurons / Q000648:ultrastructure; D009468:Neuromuscular Diseases / Q000473:pathology; D009474:Neurons / Q000648:ultrastructure; D011149:Pons / Q000648:ultrastructure
D008543:Melanins