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Postural Tremor and Ataxia Progression in Spinocerebellar Ataxias

Authors:

Shi-Rui Gan,

Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, US; Department of Neurology and Institute of Neurology, First Affiliated Hospital, Fujian Medical University, Fuzhou, CN
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Jie Wang,

Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, US; Department of Basic and Community Nursing, School of Nursing, Nanjing Medical University, Nanjing, Jiangsu, CN
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Karla P. Figueroa,

Department of Neurology, University of Utah, Salt Lake City, UT, US
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Stefan M. Pulst,

Department of Neurology, University of Utah, Salt Lake City, UT, US
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Darya Tomishon,

Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, US
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Danielle Lee,

Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, US
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Susan Perlman,

Department of Neurology, University of California, Los Angeles, CA, US
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George Wilmot,

Department of Neurology, Emory University, Atlanta, GA, US
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Christopher M. Gomez,

Department of Neurology, University of Chicago, Chicago, IL, US
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Jeremy Schmahmann,

Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, US
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Henry Paulson,

Department of Neurology, University of Michigan, Ann Arbor, MI, US
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Vikram G. Shakkottai,

Department of Neurology, University of Michigan, Ann Arbor, MI, US
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Sarah H. Ying,

Department of Neurology, Johns Hopkins University, Baltimore, MD, US
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Theresa Zesiewicz,

Department of Neurology, University of South Florida, Tampa, FL, US
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Khalaf Bushara,

Department of Neurology, University of Minnesota, Minneapolis, MN, US
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Michael D. Geschwind,

Department of Neurology, University of California San Francisco, CA, US
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Guangbin Xia,

Department of Neurology, School of Medicine, University of New Mexico, Albuquerque, NM, US
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S. H. Subramony,

Department of Neurology, School of Medicine, University of New Mexico, Albuquerque, NM, US
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Tetsuo Ashizawa,

Houston Methodist Research Institute, Houston, TX, US
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Sheng-Han Kuo

Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, US
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Abstract

Background: Postural tremor can sometimes occur in spinocerebellar ataxias (SCAs). However, the prevalence and clinical characteristics of postural tremor in SCAs are poorly understood, and whether SCA patients with postural tremor have different ataxia progression is not known.

Methods: We studied postural tremor in 315 patients with SCA1, 2, 3, and 6 recruited from the Clinical Research Consortium for Spinocerebellar Ataxias (CRC-SCA), which consists of 12 participating centers in the United States, and we evaluated ataxia progression in these patients from January 2010 to August 2012.

Results: Among 315 SCA patients, postural tremor was most common in SCA2 patients (SCA1, 5.8%; SCA2, 27.5%; SCA3, 12.4%; SCA6, 16.9%; p = 0.007). SCA3 patients with postural tremor had longer CAG repeat expansions than SCA3 patients without postural tremor (73.67 ± 3.12 vs. 70.42 ± 3.96, p = 0.003). Interestingly, SCA1 and SCA6 patients with postural tremor had a slower rate of ataxia progression (SCA1, β = –0.91, p < 0.001; SCA6, β = –1.28, p = 0.025), while SCA2 patients with postural tremor had a faster rate of ataxia progression (β = 1.54, p = 0.034).We also found that the presence of postural tremor in SCA2 patients could be influenced by repeat expansions of ATXN1 (β = –1.53, p = 0.037) and ATXN3 (β = 0.57, p = 0.018), whereas postural tremor in SCA3 was associated with repeat lengths in TBP (β = 0.63, p = 0.041) and PPP2R2B (β = –0.40, p = 0.032).

Discussion: Postural tremor could be a clinical feature of SCAs, and the presence of postural tremor could be associated with different rates of ataxia progression. Genetic interactions between ataxia genes might influence the brain circuitry and thus affect the clinical presentation of postural tremor.

How to Cite: Gan S-R, Wang J, Figueroa KP, Pulst SM, Tomishon D, Lee D, et al.. Postural Tremor and Ataxia Progression in Spinocerebellar Ataxias. Tremor and Other Hyperkinetic Movements. 2017;7:492. DOI: http://doi.org/10.7916/D8GM8KRH
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Published on 09 Oct 2017.
Peer Reviewed

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