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The Role of the Cerebellum in Huntington's Disease: a Systematic Review.

Authors
  • Franklin, Gustavo L1
  • Camargo, Carlos Henrique F2
  • Meira, Alex T3
  • Lima, Nayra S C4
  • Teive, Hélio A G3, 2
  • 1 Movement Disorders Unit, Neurology Service, Internal Medicine Department, Hospital de Clínicas, Federal University of Paraná, Rua General Carneiro 1103/102, Centro, Curitiba, Paraná, Brazil. [email protected] , (Brazil)
  • 2 Neurological Diseases Group, Graduate Program in Internal Medicine, Internal Medicine Department, Hospital de Clínicas, Federal University of Paraná, Curitiba, Paraná, Brazil. , (Brazil)
  • 3 Movement Disorders Unit, Neurology Service, Internal Medicine Department, Hospital de Clínicas, Federal University of Paraná, Rua General Carneiro 1103/102, Centro, Curitiba, Paraná, Brazil. , (Brazil)
  • 4 Vila Velha University, Vila Velha, Espírito Santo, Brazil. , (Brazil)
Type
Published Article
Journal
Cerebellum (London, England)
Publication Date
Apr 01, 2021
Volume
20
Issue
2
Pages
254–265
Identifiers
DOI: 10.1007/s12311-020-01198-4
PMID: 33029762
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

Huntington's disease (HD) is a rare neurological disorder characterized by progressive motor, cognitive, and psychiatric disturbances. Although striatum degeneration might justify most of the motor symptoms, there is an emerging evidence of involvement of extra-striatal structures, such as the cerebellum. To elucidate the cerebellar involvement and its afferences with motor, psychiatric, and cognitive symptoms in HD. A systematic search in the literature was performed in MEDLINE, LILACS, and Google Scholar databases. The research was broadened to include the screening of reference lists of review articles for additional studies. Studies available in the English language, dating from 1993 through May 2020, were included. Clinical presentation of patients with HD may not be considered as the result of an isolated primary striatal dysfunction. There is evidence that cerebellar involvement is an early event in HD and may occur independently of striatal degeneration. Also, the loss of the compensation role of the cerebellum in HD may be an explanation for the clinical onset of HD. Although more studies are needed to elucidate this association, the current literature supports that the cerebellum may integrate the natural history of neurodegeneration in HD.

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