Multisystem Degeneration of the Autonomic Nervous System

Shy Drager Syndrome

Authors

  • Pooja Butani, DO A.T. Still University, Mesa, AZ
  • Doug Fisher, DO A.T. Still University, Mesa, AZ
  • Neil Espiritu, MD The Wright Center for GME, Scranton, PA
  • Justine Carlo Guevarra, MD The Wright Center for GME, Scranton, PA
  • Mohana Partheeban, MD The Wright Center for GME, Scranton, PA
  • Nasr Simin, MD The Wright Center for GME, Scranton, PA

Keywords:

autonomic nervous system, neurological disorder

Abstract

Shy - Drager syndrome, now called Multiple System Atrophy (MSA) is a rare, degenerative neurological disorder that affects autonomic, pyramidal, parkinsonian, and cerebellar functions. [1] This condition was initially classified in 1960 for cases of hypotension of unknown etiology from neurologic disorder. Depending on predominant clinical features, MSA is categorized in MSA-P for parkinsonian and MSA-C for cerebellar. Several hypotheses have been proposed as the etiology of Multiple System Atrophy but its exact cause is yet to be discovered. This case report outlines a patient diagnosed with Shy-Drager Syndrome who is living with various autonomic symptoms that prove difficult to manage with unpredictable fluctuations. The purpose of this case report is to present a case and outline some treatment approached currently used in the management of patient with such a syndrome. 

References

Factor, S., & Doss Esper, C. (2023). Multiple system atrophy: Clinical features and diagnosis. [Review of Multiple system atrophy: Clinical features and diagnosis]. https://www.uptodate.com/contents/multiple-system-atrophy-clinical-features-and-diagnosis?search=shy%20drager%20syndrome&source=search_result&selectedTitle=1~55&usage_type=default&display_rank=1

Fecek, C. (2022). Shy Drager Syndrome [Review of Shy Drager Syndrome]. https://www.ncbi.nlm.nih.gov/books/NBK560502/

Poewe, W., Stankovic, I., Halliday, G., Meissner, W., Wenning, G., Pellecchia, M. T., Seppi, K., Palma, J.-A., & Kaufmann, H. (n.d.). Multiple system atrophy [Review of Multiple system atrophy]. https://doi.org/10.1038/s41572-022-00382-6

Fanciulli A, Stankovic I, Krismer F, Seppi K, Levin J, Wenning GK. Multiple system atrophy. Int Rev Neurobiol. 2019;149:137-192. doi:10.1016/bs.irn.2019.10.004

Ozawa T, Paviour D, Quinn NP, Josephs KA, Sangha H, Kilford L, Healy DG, Wood NW, Lees AJ, Holton JL, Revesz T. The spectrum of pathological involvement of the striatonigral and olivopontocerebellar systems in multiple system atrophy: clinicopathological correlations. Brain. 2004 Dec;127(Pt 12):2657-71. doi: 10.1093/brain/awh303. Epub 2004 Oct 27. PMID: 15509623.

Jain RS, Kumar S, Tejwani S. Medullary Hot-Cross Bun Sign in Multiple System Atrophy-Cerebellar. J Med Imaging Radiat Sci. 2016;47(1):113-115. doi:10.1016/j.jmir.2015.10.001

Stefanova N, Bücke P, Duerr S, Wenning GK. Multiple system atrophy: an update. Lancet Neurol. 2009 Dec;8(12):1172-8. doi: 10.1016/S1474-4422(09)70288-1. PMID: 19909915.

Wenning GK, Colosimo C, Geser F, Poewe W. Multiple system atrophy. Lancet Neurol. 2004 Feb;3(2):93-103. doi: 10.1016/s1474-4422(03)00662-8. Erratum in: Lancet Neurol. 2004 Mar;3(3):137. PMID: 14747001.

Downloads

Published

2024-06-30

How to Cite

Butani, P., Fisher, D., Espiritu, N., Guevarra, J. C., Partheeban, M., & Simin, N. (2024). Multisystem Degeneration of the Autonomic Nervous System: Shy Drager Syndrome. The Scholarly Society of America, 3(1), 46–49. Retrieved from http://ssajm-online.org/ojs/index.php/SSAJM/article/view/18

Issue

Vol. 3 (2024): Issue 1

Section

Special Case

License

Copyright (c) 2024 The Scholarly Society of America

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Most read articles by the same author(s)