Clinical and neuropathological findings are reported on a 48-year-old man who developed progressive bulbar palsy, muscle atrophy of four extremities, and hyperreflexia. Duration of the illness was about 19 months. Moderate personality changes were observed during his hospitalization. Neuropathological examination revealed the presence of an ALS: severe loss of the large motoneurons in the spinal anterior horns, and degeneration of the corticospinal tract, more prominent on the left side. The hypoglossal, the facial, and the motor trigeminal nuclei were also involved, but the oculomotor, the trochlear and the abducens nuclei were well preserved. There was bilateral, but more pronounced on the right, atrophy of the temporal poles consistent with the lesions of the temporal types of Pick's disease. This case indicates the simultaneous occurrence of degenerative diseases of the CNS, and the correspondence of laterality between the temporal lobe and the spinal cord may suggest a common etiology of these two types of disease processes.