Neuroaxonal dystrophy in man: character and natural history.

Abstract

A short correlative review is given of the light and electron microscopical features and histochemical reactions of dystrophic axons in the mature human CNS. Their ultrastruc- tural similarities to axonal changes in the disease entity of the neuroaxonal dystrophies and various experimental conditions are confirmed. The incidence of axonal spheroids in the gracile and cuneate nuclei and in the reticular zone of the substantia nigra was studied in 1450 consecutive autopsies and correlated with age and underlying disease process. Axonal dystrophy of posterior column nuclei and substantia nigra showed a significant age-dependancy without predilection for either sex. The incidence of dystrophic axons in the globus pallidus and other sites was also examined. No clear relationship of the severity and frequency of the involvement of gracile nucleus with any underlying disease was established, while axonal dystrophy in substantia nigra showed significant correlations to alcoholic encephalopathies and Parkinson’s syndrome, and a trend to negative relationship with presenile cerebral atrophies. In addition to the natural occurrence of dystrophic axons as a physiological, age-dependant phenomenon, these lesions may arise prematurely and to excess in a variety of natural and experimental disorders. The reported findings suggest that some metabolic factors are involved in the pathogenesis of dystrophic axonal changes. Relations to axonal dying-back processes are discussed.