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Author Notes:

Correspondence: Dr. Sheng-Han Kuo, 650 West 168th Street, Room 305, New York, NY 10032, USA. Tel: (212) 342-3753, Fax: (212) 305-1304, sk3295@columbia.edu

Author Contributions:Ms. Lai: study concept and design, statistical analysis and interpretation, writing the manuscript, critical revision of the manuscript for important intellectual content. Mrs. Tomishon: statistical analysis and interpretation, critical revision of the manuscript for important intellectual content.

Ms. Figueroa: acquisition of data, critical revision of the manuscript for important intellectual content, study supervision. Dr. Pulst: acquisition of data, critical revision of the manuscript for important intellectual content, study supervision.

Dr. Perlman: acquisition of data. Dr. Wilmot: acquisition of data. Dr. Gomez: acquisition of data. Dr. Schmahmann: acquisition of data. Dr. Paulson: acquisition of data. Dr. Shakkottai: acquisition of data. Dr. Ying: acquisition of data. Dr. Zesiewicz: acquisition of data. Dr. Bushara: acquisition of data.

Dr. Geschwind: acquisition of data. Dr. Xia: acquisition of data. Dr. Subramony: acquisition of data. Dr. Ashizawa: acquisition of data. Dr. Kuo: study concept and design, acquisition of data, analysis and interpretation, critical revision of the manuscript for important intellectual content.

Disclosure: Authors reported no conflict of interests.

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Research Funding:

The CRC-SCA natural history study is supported by the Rare Disease Clinical Research Network (RDCRN) (RC1NS068897), and the National Ataxia Foundation.

Dr. Kuo is supported by the NINDS K08 NS083738, Louis V. Gerstner Jr. Scholarship, American Brain Research Training Fellowship, Parkinson’s Disease Foundation, American Parkinson’s Disease Association, Rare Disease Clinical Research Network (RDCRN) (RC1NS068897), International Essential Tremor Foundation, and NIEHS pilot grant ES009089, the Smart Foundation.

Keywords:

  • Cerebellum
  • Neurodegeneration
  • Tremor
  • Adult
  • Aged
  • Cerebellum
  • Female
  • Humans
  • Male
  • Middle Aged
  • Spinocerebellar Ataxias
  • Tremor

Tremor in the Degenerative Cerebellum: Towards the Understanding of Brain Circuitry for Tremor

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Journal Title:

Cerebellum

Volume:

Volume 18, Number 3

Publisher:

, Pages 519-526

Type of Work:

Article | Post-print: After Peer Review

Abstract:

Background: Cerebellar degenerative pathology has been identified in tremor patients; however, how the degenerative pathology could contribute to tremor remains unclear. If the cerebellar degenerative pathology can directly drive tremor, one would hypothesize that tremor is likely to occur in the diseases of cerebellar ataxia and follows the disease progression in such disorders. Methods: To further test this hypothesis, we studied the occurrence of tremor in different disease stages of classical cerebellar degenerative disorders: spinocerebellar ataxias (SCAs). We further separately analyzed postural tremor and rest tremor, two forms of tremor that both involve the cerebellum. We also explored tremor in different subtypes of SCAs. Results: We found that 18.1% of SCA patients have tremor. Interestingly, SCA patients with tremor have worse ataxia than those without tremor. When stratifying patients into mild, moderate, and severe disease stages according to the severity of ataxia, moderate and severe SCA patients more commonly have tremor than those with mild ataxia, the effect most prominently observed in postural tremor of SCA3 and SCA6 patients. Finally, tremor can independently contribute to worse functional status in SCA2 patients, even after adjusting for ataxia severity. Conclusions: Tremor is more likely to occur in the severe stage of cerebellar degeneration when compared to mild stages. Our results partially support the cerebellar degenerative model of tremor.

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© Springer Science+Business Media, LLC, part of Springer Nature 2019.

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