MOTOR-NEURON DISEASE WITH PALLIDO-LUYSIO-NIGRAL ATROPHY

A case of motor neuron disease (MND) with pallido-luysio-nigral atroph y (PLNA) is reported. The 45-year-old male patient presented with lowe r motor neuron symptoms and signs of basal ganglia disturbance. He die d after a progressive course of 7 months. Neuropathological examinatio n revealed motor neuron loss at all spinal cord levels with sparing of Onuf's nucleus. Nerve cell loss and gliosis were also present in subs tantia nigra, globus pallidus, and subthalamic nucleus. The presence o f ubiquitin-positive inclusions, a hallmark of most variants of MND, c onfirms this case as an example of MND. At immunoelectron microscopy t he granules were distributed on filamentous material. The combination of clinically apparent PLNA with MND has only been described twice pre viously. The relationship of this syndrome to other forms of MND and i ts nosological placement are discussed.