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-luy sio-nigral system and brainstem tegmentum is presented, A 51-year-old man developed progressive muscle atrophy with fasciculation predominan tly in the shoulder girdle, upper arms, upper back, and neck in additi on to hyperreflexia and a positive Chaddock reflex. He also had retini tis pigmentosa, high arched palate, and mild hand tremor. He eventuall y developed bulbar palsy and died of paralysis of the respiratory musc les 11 years after the onset of his illness. Neuropathological examina tions showed prominent neuronal loss and gliosis in the pallido-luysio -nigral system and the tegmentum of the brainstem in addition to the s imultaneous involvement of the upper and lower motor neurons, This pat ient and 6 similar patients are discussed in relation to pallido-luysi o-nigral atrophy and the topographic distribution of degeneration in a myotrophic lateral sclerosis.