Ubiquitin-immunoreactive inclusions in anterior horn cells and hypoglossal neurons in a case with Joseph's disease.

We describe a patient with progressive spastic ataxia and ophthalmoparesis. His clinical and neuropathological findings were consistent with Joseph's disease. The most characteristic neuropathological features in the present case were ubiquitin-immunoreactive filamentous or dense inclusions in spinal anterior horn cells and hypoglossal neurons, which have been considered to be a specific finding in amyotrophic lateral sclerosis (ALS). The occurrence of ubiquitin-immunoreactive inclusions suggests that such inclusions are not totally specific to ALS and could occur in occasional degenerating motor neurons without apparent ALS neuropathology.