UPPER MOTOR-NEURON PREDOMINANT DEGENERATION WITH FRONTAL AND TEMPORAL-LOBE ATROPHY

The autopsy findings of a 78-year-old man mimicking primary lateral sc lerosis (PLS) are reported. He showed slowly progressive spasticity, p seudobulbar palsy and character change, and died 32 months after the o nset of symptoms. Autopsy revealed severe atrophy of the frontal and t emporal lobes, remarkable neuronal loss and gliosis in the precentral gyrus, left temporal lobe pole and amygdala, mild degeneration of the Ammon's hem, degeneration of the corticospinal tract, and very mild in volvement of the lower motor neurons. The anterior horn cells only occ asionally demonstrated Bunina body by cystatin-C staining, and skein-l ike inclusions by ubiquitin staining. This is a peculiar case with con comitant involvement in the motor cortex and temporal lobe in motor ne uron disease predominantly affecting the upper motor neuron.