Familial Motor Neuron Disease with Lewy Body-like Inclusions in the Substantia Nigra, the Subthalamic Nucleus, and the Globus Pallidus

In a familial case of motor neuron disease (MND), 2 unusual features were noted in the necropsy. The first was a pallidoluysonigral degeneration, observed in only 4 other cases of MND and which was here asymptomatic. The second was the presence in degenerated spinal cord anterior horns and in degenerated basal ganglia of neuronal Lewy body-like inclusions stained by antibodies against ubiquitin.