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The Cerebellum

, Volume 16, Issue 5–6, pp 938–944 | Cite as

Nonmotor Symptoms in Patients with Spinocerebellar Ataxia Type 10

  • Adriana MoroEmail author
  • Renato P. Munhoz
  • Mariana Moscovich
  • Walter O. Arruda
  • Salmo Raskin
  • Laura Silveira-Moriyama
  • Tetsuo Ashizawa
  • Hélio A. G. Teive
Original Paper

Abstract

Nonmotor symptoms (NMS) have been described in several neurodegenerative diseases but have not been systematically evaluated in spinocerebellar ataxia type 10 (SCA10). The objective of the study is to compare the frequency of NMS in patients with SCA10, Machado-Joseph disease (MJD), and healthy controls. Twenty-eight SCA10, 28 MJD, and 28 healthy subjects were prospectively assessed using validated screening tools for chronic pain, autonomic symptoms, fatigue, sleep disturbances, psychiatric disorders, and cognitive function. Chronic pain was present with similar prevalence among SCA10 patients and healthy controls but was more frequent in MJD. Similarly, autonomic symptoms were found in SCA10 in the same proportion of healthy individuals, while the MJD group had higher frequencies. Restless legs syndrome and REM sleep behavior disorder were uncommon in SCA10. The mean scores of excessive daytime sleepiness were worse in the SCA10 group. Scores of fatigue were higher in the SCA10 sample compared to healthy individuals, but better than in the MJD. Psychiatric disorders were generally more prevalent in both spinocerebellar ataxias than among healthy controls. The cognitive performance of healthy controls was better compared with SCA10 patients and MJD, which showed the worst scores. Although NMS were present among SCA10 patients in a higher proportion compared to healthy controls, they were more frequent and severe in MJD. In spite of these comparisons, we were able to identify NMS with significant functional impact in patients with SCA10, indicating the need for their systematic screening aiming at optimal treatment and improvement in quality of life.

Keywords

Spinocerebellar ataxia type 10 Machado-Joseph disease Nonmotor symptoms Spinocerebellar ataxia type 3 

Notes

Compliance with Ethical Standards

This study was approved by the institutional ethics committee of the Federal University of Paraná and all patients and healthy controls signed an informed consent.

Conflict of Interest

TA was supported by NIH grant NS083564.

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Copyright information

© Springer Science+Business Media, LLC 2017

Authors and Affiliations

  1. 1.Movement Disorders Unit, Neurology Service, Internal Medicine Department, Hospital de ClínicasFederal University of ParanáCuritibaBrazil
  2. 2.Department of Medicine, Morton and Gloria Shulman Movement Disorders Centre, Toronto Western HospitalUniversity of TorontoTorontoCanada
  3. 3.Advanced Molecular Research Center, Center for Biological and Health SciencesPUCCuritibaBrazil
  4. 4.Postgraduate Program in MedicineUniversidade Nove de JulhoSão PauloBrazil
  5. 5.Methodist Hospital Research InstituteHoustonUSA

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