Familial motor neuron disease with Lewy body-like inclusions in the substantia nigra, the subthalamic nucleus, and the globus pallidus

J Neurol Sci. 1992 Mar;108(1):18-23. doi: 10.1016/0022-510x(92)90182-k.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Genes, Dominant
  • Gliosis / etiology
  • Gliosis / pathology
  • Globus Pallidus / pathology*
  • Humans
  • Lewy Bodies* / chemistry
  • Male
  • Middle Aged
  • Motor Neuron Disease / genetics*
  • Motor Neuron Disease / pathology
  • Nerve Degeneration
  • Pedigree
  • Spinal Cord / pathology
  • Substantia Nigra / pathology*
  • Ubiquitins / analysis

Substances

  • Ubiquitins