Motor neuron disease with involvement of the pallido-luysio-nigral system and mesencephalic tegmentum.

A rare case of motor neuron disease and involvement of the pallido-luysio-nigral system and brainstem tegmentum is presented. A 51-year-old man developed progressive muscle atrophy with fasciculation predominantly in the shoulder girdle, upper arms, upper back, and neck in addition to hyperreflexia and a positive Chaddock reflex. He also had retinitis pigmentosa, high arched palate, and mild hand tremor. He eventually developed bulbar palsy and died of paralysis of the respiratory muscles 11 years after the onset of his illness. Neuropathological examinations showed prominent neuronal loss and gliosis in the pallido-luysio-nigral system and the tegmentum of the brainstem in addition to the simultaneous involvement of the upper and lower motor neurons. This patient and 6 similar patients are discussed in relation to pallido-luysio-nigral atrophy and the topographic distribution of degeneration in amyotrophic lateral sclerosis.