Temporal lobe pathology in patients with amyotrophic lateral sclerosis

Abstract

The temporal lobes of 30 clinically and pathologically confirmed patients with amyotrophic lateral sclerosis (ALS) were examined. Among them, four patients showed mild to moderate mental changes. Ten patients, including four patients with mental changes, showed ubiquitinpositive inclusions in the temporal lobes, and 12 patients showed non‐specific degenerative changes, mainly in the limbic system. Focal neuronal depletions with gliosis in the subiculum were also observed in these 12 patients, and these lesions seemed to be secondary to perforant pathway damage. The fibers from the superficial layers of the lateral parts of the entorhinal cortices (perforant pathway) were probably depleted and these changes may play an important role in mental changes in dementia with ALS. The limbic system is occasionally affected in autopsy cases of patients with ALS, and more detailed clinicopathological studies are required.