1992 Volume 29 Issue 9 Pages 644-651
It has been assumed that amyotrophic lateral sclerosis (ALS) involves precocious senility as one of its pathogenetic aspects. The authors studied 55 autopsied cases of ALS in relation to age at death, ranging from 42 to 86. The materials consisted of 8 cases in the fifth decade, 8 in the sixth, 20 in the seventh, 12 in the eighth, and 7 in the ninth. The total duration of illness ranged from 6 months to 14 years. The most distinct relationship was observed in the anterior horn lesion of the cervical enlargement which became less severe with advancing age, irrespective of the length of illness. Fifth decade cases showed marked atrophy with severe neuronal loss and fibrillary gliosis in the anterior horn, while those in the ninth decade showed slight changes which were similar to age-matched controls. On the other hand, pyramidal tract degeneration did not show any correlation to age at death or to length of illness. Pyramidal tract degeneration was found in all younger age group cases, being always severe. In the older age groups, however, the degeneration varied extremely in degree from case to case. Some cases showed severe degeneration comparable with that in the younger age groups, while the others had no findings suggesting degeneration. In addition, cases on artificial respirators had a longer duration of illness, and more marked degeneration in the anterior horn, irrespective of age. Our study did not reveal that senile changes including senile plaques and neurofibrillary tangles were more marked in ALS cases. No clinicopathological correlation with dementia was recognized. The findings suggest that the anterior horn change became less severe with age, and that normal aging in the anterior horn may play an important role in the development of disease.
