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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

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Summary

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.

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Hartmann, H.A., White, S.K. & Levine, R.L. Neuroaxonal dystrophy with neuromelanin deposition, neurofibrillary tangles, and neuronal loss. Acta Neuropathol 61, 169–172 (1983). https://doi.org/10.1007/BF00691981

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  • DOI: https://doi.org/10.1007/BF00691981

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