Onuf's nucleus is frequently involved in motor neuron disease/amyotrophic lateral sclerosis

J Neurol Sci. 1995 Apr;129(2):141-6. doi: 10.1016/0022-510x(94)00263-n.


Involvement of Onuf's nucleus (ON) in 28 cases of amyotrophic lateral sclerosis/motor neuron disease (MND/ALS) with different clinical syndromes is reported. Although significant neuronal loss was absent, all cytoskeletal abnormalities typical of alpha-motor neurons in MND/ALS were found in ON. Spheroids were detected in 53.5% of cases; 0.6-4.5% of ON neurons contained Bunina bodies, which were present in 42.8% of cases. Ubiquitin-reactive inclusions (UBRI) of filamentous and hyaline type were found in 57.1% of cases and in 1.2-10.7% of ON neurons. Cases with pyramidal tract involvement (ALS) were involved by UBRI in 76.5%, whereas cases with progressive spinal muscular atrophy revealed the same inclusions in only 27.2%. No similar inclusions were present in sacral parasympathetic intermediolateral nucleus. It can be concluded, therefore, that ON belongs to the somatic motor system and is principally vulnerable to MND/ALS, albeit to a lower degree.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Amyotrophic Lateral Sclerosis / physiopathology*
  • Humans
  • Middle Aged
  • Motor Neuron Disease / pathology
  • Motor Neuron Disease / physiopathology*
  • Motor Neurons / physiology*
  • Spinal Cord / pathology
  • Spinal Cord / physiopathology*
  • Syndrome