Unveiling the role of cerebellar alterations in the autonomic nervous system: a systematic review of autonomic dysfunction in spinocerebellar ataxias

J Neurol. 2023 Dec;270(12):5756-5772. doi: 10.1007/s00415-023-11993-8. Epub 2023 Sep 26.

Abstract

Background: Autonomic dysfunctions are prevalent in several cerebellar disorders, but they have not been systematically investigated in spinocerebellar ataxias (SCAs). Studies investigating autonomic deficits in SCAs are fragmented, with each one focusing on different autonomic dysfunctions and different SCA subtypes.

Methods: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement, we conducted a systematic review of the literature to assess the presence of autonomic dysfunctions in various SCAs. PubMed served as the primary database, and the Rayyan web application was employed for study screening.

Results: We identified 46 articles investigating at least one autonomic function in patients with SCA. The results were analyzed and categorized based on the genetic subtype of SCA, thereby characterizing the specific autonomic deficits associated with each subtype.

Conclusion: This review confirms the presence of autonomic dysfunctions in various genetic subtypes of SCA, underscoring the cerebellum's role in the autonomic nervous system (ANS). It also emphasizes the importance of investigating these functions in clinical practice.

Keywords: Autonomic dysfunctions; Cerebellar disorders; Cerebellum; Heart rate variability.

Publication types

  • Systematic Review
  • Review

MeSH terms

  • Autonomic Nervous System
  • Cerebellum
  • Humans
  • Primary Dysautonomias*
  • Spinocerebellar Ataxias* / genetics