Which histologic findings suggest autonomic failure syndromes?

Updated: Oct 21, 2018
  • Author: Mohini Gurme, MD; Chief Editor: Selim R Benbadis, MD  more...
  • Print
Answer

Biopsy of the CNS is never part of the routine evaluation for these disorders (see Procedures). However, brain autopsy specimens in MSA show distinct glial cytoplasmic inclusions composed of 20- to 30-nm multilayered tubular filaments that are argyrophilic. The inclusions are found in the basal ganglia, the supplementary and primary motor cortex, the reticular formation, and the pontocerebellar system.

Alpha-synuclein is present in the glial inclusions and appears to play an important role in MSA. The autonomic failure in MSA likely results from cell loss in the dorsal motor nucleus of vagus nerve, locus coeruleus, and the catecholaminergic neurons of the ventrolateral medulla. Cell loss in the pontomedullary reticular formation, parasympathetic preganglionic nuclei of the spinal cord, and sympathetic intermediolateral column of the spinal cord are also important.

Other limited data on PAF demonstrate additional nerve cell loss and Lewy bodies, which stain for ubiquitin in the paravertebral sympathetic ganglia. Whether these patients had a form fruste of MSA is unclear.


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!