Quality of Life in Sporadic Adult-Onset Ataxia

  • M. Abele
Reference work entry


 Sporadic adult-onset ataxia (SAOA) is a usually slowly progressive disorder that accounts for a considerable part of the neurodegenerative cerebellar diseases. Patients with SAOA suffer from a predominant cerebellar syndrome with ataxia of stance and gait, irregular limb movements, dysarthria, and oculomotor abnormalities. In addition, extracerebellar features like disturbances of the peripheral or autonomic nervous system but also sleep related complaints and depression are frequently found. The impact of progressive motor disability and non-motor complaints on health-related quality of life (Hr-QoL) has only been studied in one small sample of german SAOA patients by means of the medical outcome study short form (SF-36). Compared to a large german control group, SAOA patients had lower scores in all SF-36 dimensions except for bodily pain. The greatest differences were seen in the domains physical functioning, followed by social functioning, role limitations (emotional problems), and general health perception. There was a significant negative correlation of all non-motor SF-36 dimensions with a depression score. Walking aid dependency was significantly correlated with poorer health status perception in several motor and non-motor domains. In addition, impaired sleep quality was correlated with an impaired general health perception and with bodily pain. The available data demonstrate a great impact of motor and non-motor symptoms of SAOA on Hr-QoL. Adequate treatment of depression, motor disability, and impaired sleep quality is therefore essential to improve Hr-QoL in ataxic patients.


Sleep Quality Multiple System Atrophy Epworth Sleepiness Scale Pittsburgh Sleep Quality Index General Health Perception 

List of Abbreviations:


aquired immunodeficiency syndrome


Beck depression inventory


bodily pain


cerebrospinal fluid


 excessive daytime sleepiness


standardised instrument for use as a measure of health outcome


Epworth sleepiness scale


Friedreich ataxia


general health perception


human immunodeficiency virus


health-related quality of life


heart rate variability


mental health


magnetic resonance imaging


 multiple system atrophy


Parkinson’s disease


physical functioning


Pittsburgh sleep quality index


role limitations (emotional problems)


role limitations (physical problems)


sporadic adult-onset ataxia


spinocerebellar ataxia


spinocerebellar ataxia type 6


standard deviation


somatosensory evoked potential


social functioning


medical outcome study short form




  1. Abdo WF, Warrenburg BP, van de Munneke M, van Geel WJ, Bloem BR, Kremer HP, Verbeek MM. (2006). Neurology. 67: 474–479.PubMedCrossRefGoogle Scholar
  2. Abele M, Burk K, Laccone F, Dichgans J, Klockgether T. (2001). J Neurol. 248: 311–314.PubMedCrossRefGoogle Scholar
  3. Abele M, Burk K, Schols L, Schwartz S, Besenthal I, Dichgans J, Zuhlke C, Riess O, Klockgether T. (2002). Brain. 125: 961–968.PubMedCrossRefGoogle Scholar
  4. Abele M, Klockgether T. (2007). Mov Disord. 22: 348–352.PubMedCrossRefGoogle Scholar
  5. Abele M, Minnerop M, Urbach H, Specht K, Klockgether T. (2007). J Neurol. 254: 1384–1389.PubMedCrossRefGoogle Scholar
  6. Beck AT, Beamesderfer A. (1974). Mod Probl Pharmacopsychiatry. 7: 151–169.PubMedGoogle Scholar
  7. Benrud-Larson LM, Sandroni P, Schrag A, Low PA. (2005). Mov Disord. 20: 951–957.PubMedCrossRefGoogle Scholar
  8. Berent S, Giordani B, Gilman S, Trask CL, Little RJ, Johanns JR, Junck L, Kluin KJ, Heumann M, Koeppe RA. (2002). Brain Cogn. 50: 194–206.Google Scholar
  9. Bullinger M. (1996). Rehabilitation (Stuttg) 35: XVII–XXVII; quiz XXVII–XXIX.Google Scholar
  10. Burk K, Buhring U, Schulz JB, Zuhlke C, Hellenbroich Y, Dichgans J. (2005). Arch Neurol. 62: 981–985.PubMedCrossRefGoogle Scholar
  11. Burk K, Globas C, Wahl T, Buhring U, Dietz K, Zuhlke C, Luft A, Schulz JB, Voigt K, Dichgans J. (2004). Brain. 127: 175–181.PubMedCrossRefGoogle Scholar
  12. Buysse DJ, Reynolds CFr, Monk TH, Berman SR, Kupfer DJ. (1989). Psychiatry Res. 28: 193–213.PubMedCrossRefGoogle Scholar
  13. Cecchin CR, Pires AP, Rieder CR, Monte TL, Silveira I, Carvalho T, Saraiva-Pereira ML, Sequeiros J, Jardim LB. (2007). Community Genet. 10: 19–26.PubMedCrossRefGoogle Scholar
  14. Ellert U, Bellach BM (1999). Gesundheitswesen 61 Spec No: S184–90.Google Scholar
  15. Fabbrini G, Barbanti P, Aurilia C, Vanacore N, Pauletti C, Meco G. (2002). Mov Disord. 17: 1026–1030.PubMedCrossRefGoogle Scholar
  16. Ghorayeb I, Bioulac B, Tison F. (2005). J Neural Transm. 112: 1669–1675.PubMedCrossRefGoogle Scholar
  17. Gilman S, Low PA, Quinn N, Albanese A, Ben-Shlomo Y, Fowler CJ, Kaufmann H, Klockgether T, Lang AE, Lantos PL, Litvan I, Mathias CJ, Oliver E, Robertson D, Schatz I, Wenning GK. (1999). J Neurol Sci. 163: 94–98.PubMedCrossRefGoogle Scholar
  18. Harding AE. (1981). J Neurol Sci. 51: 259–271.PubMedCrossRefGoogle Scholar
  19. Howell MJ, Mahowald MW, Gomez CM. (2006). Neurology. 66: 1430–1431.PubMedCrossRefGoogle Scholar
  20. Johns MW (1991). Sleep. 14: 540–545.PubMedGoogle Scholar
  21. Klockgether T, Schroth G, Diener HC, Dichgans J. (1990). J Neurol Neurosurg Psychiatry. 53: 297–305.PubMedCrossRefGoogle Scholar
  22. Lopez-Bastida J, Perestelo-Perez L, Monton-Alvarez F, Serrano-Aguilar P. (2008). Mov Disord. 23: 212–217.PubMedCrossRefGoogle Scholar
  23. Marie P, Foix C, Alajouanine T. (1922). Revue neurologique. 38: 849–885, 1082–1111.Google Scholar
  24. Muzaimi MB, Thomas J, Palmer-Smith S, Rosser L, Harper PS, Wiles CM, Ravine D, Robertson NP. (2004). J Neurol Neurosurg Psychiatry. 75: 1129–1134.PubMedCrossRefGoogle Scholar
  25. Ogawa M. (2004). Cerebellum. 3: 107–111.PubMedCrossRefGoogle Scholar
  26. Ota S, Tsuchiya K, Anno M, Niizato K, Akiyama H. (2008). Neuropathology. 28: 43–50.PubMedCrossRefGoogle Scholar
  27. Papp MI, Lantos PL. (1994). Brain. 117: 235–243.PubMedCrossRefGoogle Scholar
  28. Paus S, Brecht HM, Koster J, Seeger G, Klockgether T, Wullner U. (2003). Mov Disord. 18: 659–667.PubMedCrossRefGoogle Scholar
  29. Quinn N. (1989). J Neurol Neurosurg Psychiatry. Suppl: 78–89.Google Scholar
  30. Schrag A, Geser F, Stampfer-Kountchev M, Seppi K, Sawires M, Kollensperger M, Scherfler C, Quinn N, Pellecchia MT, Barone P, Del Sorbo F, Albanese A, Ostergaard K, Dupont E, Cardozo A, Tolosa E, Nilsson CF, Widner H, Lindvall O, Giladi N, Gurevich T, Daniels C, Deuschl G, Coelho M, Sampaio C, Abele M, Klockgether T, Schimke N, Eggert KM, Oertel W, Djaldetti R, Colosimo C, Meco G, Poewe W, Wenning GK. (2006). Mov Disord. 21: 809–815.PubMedCrossRefGoogle Scholar
  31. Schrag A, Jahanshahi M, Quinn N. (2000a). Mov Disord. 15: 1112–1118.PubMedCrossRefGoogle Scholar
  32. Schrag A, Jahanshahi M, Quinn N. (2000b). J Neurol Neurosurg Psychiatry. 69: 308–312.PubMedCrossRefGoogle Scholar
  33. Schulz JB, Klockgether T, Petersen D, Jauch M, Muller-Schauenburg W, Spieker S, Voigt K, Dichgans J. (1994). J Neurol Neurosurg Psychiatry. 57: 1047–1056.PubMedCrossRefGoogle Scholar
  34. Thorpy MJ, Adler CH. (2005). Neurol Clin. 23: 1187–1208.PubMedCrossRefGoogle Scholar
  35. Tuin I, Voss U, Kang JS, Kessler K, Rub U, Nolte D, Lochmuller H, Tinschert S, Claus D, Krakow K, Pflug B, Steinmetz H, Auburger G. (2006). Neurology. 67: 1966–1972.PubMedCrossRefGoogle Scholar
  36. Ware JEJ, Sherbourne CD. (1992). Med Care. 30: 473–483.PubMedCrossRefGoogle Scholar
  37. Wilson CL, Fahey MC, Corben LA, Collins VR, Churchyard AJ, Lamont PJ, Delatycki MB. (2007). Eur J Neurol. 14: 1040–1047.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media LLC 2010

Authors and Affiliations

  • M. Abele
    • 1
  1. 1.Department of NeurologyUniversity of BonnBonnGermany

Personalised recommendations