Shy-Drager syndrome and amyotrophic lateral sclerosis. Cytoarchitectonic and morphometric studies of sacral autonomic neurons

J Neurol Sci. 1986 Apr;73(2):193-204. doi: 10.1016/0022-510x(86)90130-9.


In order to elucidate the morphological correlates of bladder-rectal dysfunctions in Shy-Drager syndrome, the sacral spinal cord was cytoarchitectonically studied and 3 groups of sacral motor neurons, the posterolateral motor neuron column (PL), inferior intermediolateral nucleus (IML) and cell group X of Onuf (Onuf), were morphometrically quantitated at the S3 level (5 cases), after which the results were compared with those from amyotrophic lateral sclerosis (5 cases) and an age-matched control group (4 cases). The sacral autonomic preganglionic nucleus of IML was localized chiefly in the S3-4 segments and was maximally developed in the caudal one-third of S3. The cell group X of Onuf was localized between the middle of S2 and the rostral one-third of S3 as a longitudinal slender column in the ventral horn. Between these two nuclei at the rostral S3 level, a connecting cellular bridge of neurons of intermediolateral cell type was identified. Morphometry disclosed a marked deprivation of IML, Onuf and somatic motor neurons in Shy-Drager syndrome and a severe loss of somatic motor neurons and a modest deprivation of IML neurons in ALS. These results imply that these two disorders distinguished by different clinical manifestations share a common loss of somatic motor and parasympathetic motor neurons at least in the sacral cord. There are, however, certain gradients in the severity of involvement in these heterogeneous cell groups.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyotrophic Lateral Sclerosis / pathology*
  • Autonomic Fibers, Preganglionic / pathology
  • Autonomic Nervous System / pathology*
  • Autonomic Nervous System Diseases / pathology*
  • Cell Count
  • Female
  • Humans
  • Male
  • Middle Aged
  • Motor Neurons / pathology
  • Rectum / innervation
  • Shy-Drager Syndrome / pathology*
  • Spinal Cord / pathology*
  • Urinary Bladder / innervation