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The Cerebellum

, Volume 16, Issue 1, pp 118–121 | Cite as

Fatigue and Its Associated Factors in Spinocerebellar Ataxia Type 3/Machado-Joseph Disease

  • Alberto R. M. Martinez
  • Marcelo B. Nunes
  • Ingrid Faber
  • Anelyssa D’Abreu
  • Íscia Lopes-Cendes
  • Marcondes C. FrançaJrEmail author
Original Paper

Abstract

Fatigue has been described in several neurodegenerative diseases, reducing quality of life. A systematic evaluation of this clinical feature is lacking in SCA3/MJD. The aim of this study was to evaluate the frequency and the factors associated with fatigue in SCA3/MJD. Patients with SCA3/MJD and matched healthy controls answered the Modified Fatigue Impact Scale (MFIS), Beck Inventory Depression (BDI) and Epworth Sleepiness Scale (ESS). Scale for the assessment and rating of ataxia (SARA) was used to determine ataxia severity. We used Mann-Whitney and Fisher exact tests to compare mean scores and proportions between groups. Linear regression analyses were employed to investigate factors associated with fatigue in SCA3/MJD. Seventy-four patients were included with a mean age and disease duration of 47.2 ± 12.8 and 9.5 ± 6.37 years, respectively. There were 38 men and 36 women. Mean (CAG)n was 72.2 ± 3.8. Mean MFIS score was higher in patients with SCA3/MJD (41.4 ± 16.2 vs 18.4 ± 12.9, p < 0.001). According to BDI scores, relevant depressive symptoms were found in 69.4 % of patients but only in 10.4 % of controls (p < 0.001). The proportion of patients with ESS scores indicating excessive daytime somnolence was also higher than controls (37.5 vs 22.3 %, p = 0.05). In the multiple regression analysis, both BDI and ESS scores were associated with fatigue (r = 0.67, p < 0.001 and p = 0.01). Fatigue is frequent and strongly associated with depression and excessive daytime somnolence in SCA3/MJD.

Keywords

Machado-Joseph disease SCA 3 Fatigue Non-motor Depression 

Notes

Compliance with Ethical Standards

A local ethical committee approved the investigation protocol, and all patients and controls signed a voluntary informed consent form.

Conflict of Interest

The authors declare that they have no conflict of interest.

References

  1. 1.
    Kieling C, Prestes PR, Saraiva-Pereira ML, Jardim LB. Survival estimates for patients with Machado–Joseph disease (SCA3). Clin Genet. 2007;72:543–5.CrossRefPubMedGoogle Scholar
  2. 2.
    Pedroso JL, França Jr MC, Braga-Neto P, D’Abreu A, Saraiva-Pereira ML, Saute JA, et al. Nonmotor and extracerebellar features in Machado-Joseph disease: a review. Mov Disord. 2013;28:1200–8.CrossRefPubMedGoogle Scholar
  3. 3.
    Chaudhuri A, Behan PO. Fatigue in neurological disorders. Lancet. 2004;363:978–88.CrossRefPubMedGoogle Scholar
  4. 4.
    Friedman JH, Amick MM. Fatigue and daytime somnolence in Machado Joseph disease (spinocerebellar ataxia type 3). Mov Disord. 2008;23:1323–4.CrossRefPubMedGoogle Scholar
  5. 5.
    Brusse E, Brusse-Keizer MG, Duivenvoorden HJ, van Swieten JC. Fatigue in spinocerebellar ataxia, patient self-assessment of an early and disabling symptom. Neurology. 2011;76:953–9.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Gorenstein C, Andrade L. Validation of a Portuguese version of the Beck Depression Inventory and State-Tract Anxiety Inventory in Brazilian subjects. Braz J Med Biol Res. 1996;29:453–7.PubMedGoogle Scholar
  7. 7.
    Bertolazi AN, Fagondes SC, Hoff LS, Pedro VD, Menna Barreto SS, Johns MW. Portuguese-language version of Epworth sleepiness scale: validation for use in Brazil. J Bras Pneumol. 2009;35:877–83.CrossRefPubMedGoogle Scholar
  8. 8.
    Braga-Neto P, Godeiro-Junior C, Almeida LD, Pedroso JL, Barsottini OGP. Translation and validation into Brazilian version of the scale of the assessment and rating of ataxia (SARA). Arq Neuropsiquiatr. 2010;68:228–30.CrossRefPubMedGoogle Scholar
  9. 9.
    Martins Jr CR, Martinez AR, D’Abreu A, Lopes-Cendes I, França Jr MC. Fatigue is frequent and severe in spinocerebellar ataxia type 1. Parkinsonism Relat Disord. 2015;21:821–2.CrossRefPubMedGoogle Scholar
  10. 10.
    da Silva CB, Chevis CF, D’Abreu A, Lopes-Cendes I, França Jr MC. Fatigue is frequent and multifactorial in Friedreich’s ataxia. Parkinsonism Relat Disord. 2013;19:766–7.CrossRefPubMedGoogle Scholar
  11. 11.
    Servelhere KR, Faber I, Saute JA, Moscovich M, D’Abreu A, Jardim LB, Teive HA, Lopes-Cendes I, Franca MC Jr. Non-motor symptoms in patients with hereditary spastic paraplegia caused by SPG4 mutations. Eur J Neurol. 2016;23:408–11.Google Scholar
  12. 12.
    Friedman JH, Abrantes A, Sweet LH. Fatigue in Parkinson’s disease. Expert Opin Pharmacother. 2011;12:1999–2007.CrossRefPubMedGoogle Scholar
  13. 13.
    Chaudhuri A, Behan PO. Fatigue and basal ganglia. J Neurol Sci. 2000;179:34–42.CrossRefPubMedGoogle Scholar
  14. 14.
    de Rezende TJ, D’Abreu A, Guimarães RP, Lopes TM, Lopes-Cendes I, Cendes F, et al. Cerebral cortex involvement in Machado-Joseph disease. Eur J Neurol. 2015;22:277–83.CrossRefPubMedGoogle Scholar
  15. 15.
    Braga-Neto P, Felicio AC, Hoexter MQ, Pedroso JL, Dutra LA, Alessi H, et al. Cognitive and olfactory deficits in Machado-Joseph disease: a dopamine transporter study. Parkinsonism Relat Disord. 2012;18:854–8.CrossRefPubMedGoogle Scholar
  16. 16.
    França Jr MC, D’Abreu A, Nucci A, Cendes F, Lopes-Cendes I. Prospective study of peripheral neuropathy in Machado-Joseph disease. Muscle Nerve. 2009;40:1012–8.CrossRefGoogle Scholar
  17. 17.
    Yabe I, Tha KK, Yokota T, Sato K, Soma H, Takei A, et al. Estimation of skeletal muscle energy metabolism in Machado-Joseph disease using (31)P-MR spectroscopy. Mov Disord. 2011;26:165–8.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  • Alberto R. M. Martinez
    • 1
  • Marcelo B. Nunes
    • 1
  • Ingrid Faber
    • 1
  • Anelyssa D’Abreu
    • 1
  • Íscia Lopes-Cendes
    • 2
  • Marcondes C. FrançaJr
    • 1
    Email author
  1. 1.Department of NeurologyUniversity of Campinas (UNICAMP)CampinasBrazil
  2. 2.Department of Medical GeneticsUniversity of Campinas (UNICAMP)CampinasBrazil

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