Abstract
The gross and light-microscopic findings at autopsy of 2 new cases of Hallervorden-Spatz disease (HSD) of early onset and/or infantile neuroaxonal dystrophy (IND) are presented. Electron-microscopic detail of mineral pigment deposits and dystrophic axons (“spheroids”) proved to be indistinguishable in the 2 cases. The presence of numerous Lewy bodies in the substantia nigra of 1 of the cases was an unusual finding. In the same case, the ultrastructure of cytoplasmic inclusions in nerve cells of the cerebral cortex resembled the Lewy body. An analysis of the striking similarities and differences between these 2 cases and a review of previous reports lead to the conclusion that present knowledge does not permit a clear distinction between HSD of early onset and IND.
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