ADULT METACHROMATIC LEUCODYSTROPHY: CLINICOPATHOLOGICAL REPORT OF TWO FAMILIAL CASES WITH SLOW COURSE

Publisher Website
Google Scholar
Abstract
Cases (2) of adult metachromatic leucodystrophy in 1 family are reported. The main clinical features in both were predominantly psychiatric with alcoholism and an extremely long duration of the illness. Neuropathological examination revealed a similar distribution of the lesions in both, and scanty metachromatic accumulation in the CNS and not at all elsewhere. The great variability of the length of the courses is stressed. The duration in no way seems to be linked to the age of onset, except in the typical infantile form. Different lengths of history may correlate with distinct neuropathological findings, and may possibly be related to qualitative differences in the involved enzyme [arylsulfatase-A] disturbance. The classification based on the age of onset should be enlarged with a further one distinguishing between rapid and slow course types.