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They subsequently developed extrapyramidal symptoms, dementia, cerebellar ataxia, and corticospinal dysfunction, but neither demonstrated myoclonic seizures. In one patient the diagnosis was made before death by brain biopsy. On pathological examination both patients showed a generalized distribution of spheroids within the central nervous system and, in one, in the peripheral nerves. In both patients, Lewy bodies were demonstrated in the pigmented brainstem nuclei. In addition, one patient showed ultrastructurally verified Lewy bodies in the cerebral cortex. Although these two cases could be considered examples of juvenile neuroaxonal dystrophy, we prefer to regard them as transitional forms in a disease spectrum with classic infantile neuroaxonal dystrophy and Hallervorden\u2010Spatz disease at the two extremes.<\/jats:p>","DOI":"10.1002\/ana.410110403","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T13:30:35Z","timestamp":1104586235000},"page":"335-343","source":"Crossref","is-referenced-by-count":31,"title":["Neuroaxonal dystrophy in young adults: A clinicopathological study of two unrelated cases"],"prefix":"10.1002","volume":"11","author":[{"given":"Keith","family":"Williamson","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anders, A. 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