title: Fatigue in spinocerebellar ataxia: patient self-assessment of an early and disabling symptom.
abstract: OBJECTIVE
  To identify the prevalence and severity of fatigue and predicting factors for severe fatigue in autosomal dominant spinocerebellar ataxia (SCA).
  
  
  METHODS
  We studied a cross-section of 123 patients with SCA. Six functional scales were used in a self-assessment: the Fatigue Severity Scale (FSS); the Beck Depression Inventory (BDI); the Rotterdam Handicap Scale (RHS); the Short Form-36 health survey, distinguishing a norm-based physical and mental component score (Nb-PCS and Nb-MCS); the Pittsburgh Sleep Quality Index (PSQI); and the Epworth Sleepiness Scale (ESS). A subset of 58 patients was clinically evaluated, measuring severity of ataxia with the Scale for the Assessment and Rating of Ataxia and cognitive functioning with the Mini-Mental State Examination.
  
  
  RESULTS
  Severe fatigue (FSS ≥5) was present in 69% of patients and FSS value correlated with the scores on RHS, Nb-PCS, Nb-MCS, BDI, PSQI, and ESS. There was no relation with disease duration, gender, or medication use. Multivariate analysis revealed that Nb-PCS and BDI were the best independent predictors for severe fatigue. Interestingly, the presence of visual symptoms was related to FSS value in the clinically evaluated subgroup.
  
  
  CONCLUSIONS
  Fatigue is a severe and disabling symptom in adult patients with SCA, even early in the course of disease. Physical functioning and depression are the strongest predictors of fatigue. In treatment strategies, all treatable factors for fatigue should be addressed, especially depression, visual symptoms, and sleeping disorders.
authors.affiliation: Department of Neurology, Erasmus MC University Medical Center Rotterdam, PO Box 2040, 3000 CA, Rotterdam, The Netherlands. e.brusse.1@erasmusmc.nl
authors.forename: Esther
authors.initials: E
authors.lastname: Brusse
authors.forename: Marjolein G J
authors.initials: MG
authors.lastname: Brusse-Keizer
authors.forename: Hugo J
authors.initials: HJ
authors.lastname: Duivenvoorden
authors.forename: John C
authors.initials: JC
authors.lastname: van Swieten
mesh_terms: D000328:Adult; D000368:Aged; D059026:Diagnostic Self Evaluation; D005221:Fatigue / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology; D005260:Female; D006801:Humans; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D011569:Psychiatric Status Rating Scales; D012720:Severity of Illness Index; D020754:Spinocerebellar Ataxias / Q000150:complications* / Q000503:physiopathology; D011795:Surveys and Questionnaires