The Cerebellum

, Volume 10, Issue 2, pp 291–295 | Cite as

Sleep Disorders in Machado–Joseph Disease: Frequency, Discriminative Thresholds, Predictive Values, and Correlation with Ataxia-Related Motor and Non-Motor Features

  • José Luiz PedrosoEmail author
  • Pedro Braga-Neto
  • André Carvalho Felício
  • Lívia Almeida Dutra
  • William A. C. Santos
  • Gilmar Fernandes do Prado
  • Orlando Graziani Povoas Barsottini


Sleep disorders are common complaints in patients with neurodegenerative diseases such as spinocerebellar ataxia type 3 (SCA3) or Machado–Joseph disease (MJD)—SCA3/MJD. We evaluated the frequency of sleep disorders in SCA3/MJD patients against controls matched by age and gender, and correlated data with demographic and clinical variables. The main sleep disorders evaluated were rapid eye movement (REM) sleep behavior disorder (RBD), restless leg syndrome (RLS), and excessive daytime sleepiness (EDS). We recruited 40 patients with clinical and molecular-proven SCA3/MJD and 38 controls. We used the following clinical scales to evaluate our primary outcome measures: RBD Screening Questionnaire, International RLS Rating Scale, and Epworth Sleepiness Scale. To evaluate ataxia-related motor and non-motor features, we applied the International Cooperative Ataxia Rating Scale, the Scale for the Assessment and Rating of Ataxia, and the Unified Parkinson’s Disease Rating Scale part III. Psychiatric manifestations were tested with the Hamilton Anxiety Scale, and Beck Depression Inventory. The frequency of RBD and RLS were significantly higher in the SCA3/MJD group than in the control group (p < 0.001). There was no difference between both groups with regard to EDS. The accuracy of RDBSQ to discriminate between cases and controls was considered the best area under the ROC curve (0.86). Within-SCA3/MJD group analysis showed that anxiety and depression were significantly correlated with RDB, but not with RLS. Additionally, depression was considered the best predictive clinical feature for RDB and EDS.


Machado–Joseph disease Spinocerebellar ataxia type 3 Sleep disorders REM sleep behavior disorder Restless legs syndrome 



Dr Andre C. Felicio is supported by a doctorate fellowship grant from the “Fundação de Amparo à Pesquisa de São Paulo” (FAPESP). Dr. Gilmar F. Prado is partially supported by the “Conselho Nacional de Desenvolvimento Científico e Tecnológico” (CNPq).

Conflict of interest

We have no conflicts of interest.

Protection of Human Research Subjects and Human Subject Informed Consent

The procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national). All subjects were provided with the approved informed consent.


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Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • José Luiz Pedroso
    • 1
    Email author
  • Pedro Braga-Neto
    • 1
  • André Carvalho Felício
    • 1
  • Lívia Almeida Dutra
    • 1
  • William A. C. Santos
    • 1
  • Gilmar Fernandes do Prado
    • 1
  • Orlando Graziani Povoas Barsottini
    • 1
  1. 1.Department of Neurology and NeurosurgeryUniversidade Federal de São PauloSão PauloBrazil

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