| Abstract: | We report on a case of sporadic amyotrophic lateral sclerosis (ALS) with prolonged, respirator-assisted survival and clinical signs limited to the upper and lower motor neuron systems. Autopsy revealed degeneration of the upper and lower neuron systems and Bunina bodies, both characteristic of ALS. In addition, we found degeneration of the spinocerebellar tracts, the substantia nigra and the dentatorubral system. Our case is an additional example of multisystem degeneration in ALS associated with prolonged, respirator-assisted survival. These findings suggest that sporadic ALS comprises heterogeneous subgroups with patterns of degeneration that depend, in part, on survival time. |
