The Cerebellum

, Volume 9, Issue 2, pp 232–239

Falls in Spinocerebellar Ataxias: Results of the EuroSCA Fall Study

  • Ella M. R. Fonteyn
  • Tanja Schmitz-Hübsch
  • Carla C. Verstappen
  • Laslo Baliko
  • Bastiaan R. Bloem
  • Silvia Boesch
  • Lisa Bunn
  • Perrine Charles
  • Alexandra Dürr
  • Allesandro Filla
  • Paola Giunti
  • Christoph Globas
  • Thomas Klockgether
  • Bela Melegh
  • Massimo Pandolfo
  • Anna De Rosa
  • Ludger Schöls
  • Dagmar Timmann
  • Marten Munneke
  • Berry P. H. Kremer
  • Bart P. C. van de Warrenburg
Article

Abstract

To investigate the frequency, details, and consequences of falls in patients with autosomal dominant spinocerebellar ataxias (SCAs) and to derive specific disease-related risk factors that are associated with an increased fall frequency. Two hundred twenty-eight patients with SCA1, SCA2, SCA3, or SCA6, recruited from the EuroSCA natural history study, completed a fall questionnaire that assessed the frequency, consequences, and several details of falls in the previous 12 months. Relevant disease characteristics were retrieved from the EuroSCA registry. The database of the natural history study provided the ataxia severity scores as well as the number and nature of non-ataxia symptoms. Patients (73.6%) reported at least one fall in the preceding 12 months. There was a high rate of fall-related injuries (74%). Factors that were associated with a higher fall frequency included: disease duration, severity of ataxia, the presence of pyramidal symptoms, the total number of non-ataxia symptoms, and the genotype SCA3. Factors associated with a lower fall frequency were: the presence of extrapyramidal symptoms (more specifically dystonia of the lower limbs) and the genotype SCA2. The total number of non-ataxia symptoms and longer disease duration were independently associated with a higher fall frequency in a logistic regression analysis, while the presence of extrapyramidal symptoms was independently associated with a lower fall frequency. Our findings indicate that, in addition to more obvious factors that are associated with frequent falls, such as disease duration and ataxia severity, non-ataxia manifestations in SCA play a major role in the fall etiology of these patients.

Keywords

Ataxia SCA Falls Pyramidal Injuries 

Supplementary material

12311_2010_155_MOESM1_ESM.doc (48 kb)
ESM 1Dutch Questionnaire (DOC 48 kb)
12311_2010_155_MOESM2_ESM.doc (54 kb)
ESM 2English Questionnaire (DOC 54 kb)
12311_2010_155_MOESM3_ESM.doc (52 kb)
ESM 3French Questionnaire (DOC 52 kb)
12311_2010_155_MOESM4_ESM.doc (48 kb)
ESM 4German Questionnaire (DOC 47 kb)

References

  1. 1.
  2. 2.
    Bloem BR, Boers I, Cramer M, Westendorp RG, Gerschlager W (2001) Falls in the elderly. I. Identification of risk factors. Wien Klin Wochenschr 113:352–362PubMedGoogle Scholar
  3. 3.
    Vellas BJ, Wayne SJ, Romero LJ, Baumgartner RN, Garry PJ (1997) Fear of falling and restriction of mobility in elderly fallers. Age Ageing 26:189–193CrossRefPubMedGoogle Scholar
  4. 4.
    Zijlstra GA, van Haastregt JC, van Eijk JT, van Rossum E, Stalenhoef PA, Kempen GI (2007) Prevalence and correlates of fear of falling, and associated avoidance of activity in the general population of activity in the general population of community-living older people. Age Ageing 36:304–309CrossRefPubMedGoogle Scholar
  5. 5.
    Van de Warrenburg BPC, Steijns JA, Munneke M, Kremer HP, Bloem BR (2005) Falls in degenerative cerebellar ataxias. Mov Disord 20:497–508CrossRefPubMedGoogle Scholar
  6. 6.
    Schmitz-Hübsch T, du Montcel ST, Baliko L et al (2006) Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology 66:1717–1720CrossRefPubMedGoogle Scholar
  7. 7.
    Klockgether T, Lüdtke R, Kramer B et al (1998) The natural history of degenerative ataxia: a retrospective study in 466 patients. Brain 121:589–600CrossRefPubMedGoogle Scholar
  8. 8.
    Lynch DR, Farmer JM, Tsou AJ et al (2006) Measuring Friedreich ataxia: complementary features of examination and performance measures. Neurology 66:1711–1716CrossRefPubMedGoogle Scholar
  9. 9.
    Bloem BR, Munneke M, Mazibrada G et al (2004) The nature of falling in progressive supranuclear palsy. Mov Disord 19:359–360CrossRefGoogle Scholar
  10. 10.
    Bloem BR, Grimbergen YAM, Cramer M, Willemsen M, Zwinderman AH (2001) Prospective assessment of falls in Parkinson’s disease. J Neurol 248:950–958CrossRefPubMedGoogle Scholar
  11. 11.
    Bloem BR, Mazibrada G, Schrag A et al (2002) Falls and injuries in progressive supranuclear palsy (PSP). Mov Disord 17(Suppl):252Google Scholar
  12. 12.
    Pieterse AJ, Luttikhold TB, De Laat B, Bloem BR, Van Engelen BG, Munneke M (2006) Falls in patients with neuromuscular disorders. J Neurol 251:87–90Google Scholar
  13. 13.
    Stolze H, Klebe S, Zechlin C, Baecker C, Friege L, Deuschl G (2004) Falls in frequent neurological diseases. J Neurol 251:79–84CrossRefPubMedGoogle Scholar
  14. 14.
    Van de Warrenburg BPC, Bakker M, Kremer HP, Bloem BR, Allum JHJ (2005) Trunk sway in patients with spinocerebellar ataxia. Mov Disord 20:1006–1013CrossRefPubMedGoogle Scholar
  15. 15.
    Stolze H, Friedrich HJ, Steinauer K, Vieregge P (2000) Stride parameters in healthy young and old women -measurement variability on a simple walkway. Exp Aging Res 26:159–168CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Ella M. R. Fonteyn
    • 1
  • Tanja Schmitz-Hübsch
    • 2
  • Carla C. Verstappen
    • 3
  • Laslo Baliko
    • 4
  • Bastiaan R. Bloem
    • 1
  • Silvia Boesch
    • 5
  • Lisa Bunn
    • 6
  • Perrine Charles
    • 7
    • 8
  • Alexandra Dürr
    • 7
    • 8
  • Allesandro Filla
    • 9
  • Paola Giunti
    • 6
  • Christoph Globas
    • 10
  • Thomas Klockgether
    • 2
  • Bela Melegh
    • 11
  • Massimo Pandolfo
    • 12
  • Anna De Rosa
    • 9
  • Ludger Schöls
    • 10
  • Dagmar Timmann
    • 13
  • Marten Munneke
    • 1
  • Berry P. H. Kremer
    • 14
  • Bart P. C. van de Warrenburg
    • 1
    • 15
  1. 1.Department of Neurology (935)Radboud University Nijmegen Medical CentreNijmegenThe Netherlands
  2. 2.Department of NeurologyUniversity Hospital of BonnBonnGermany
  3. 3.Department of NeurologyCanisius Wilhelmina HospitalNijmegenThe Netherlands
  4. 4.Department of Neurology and StrokeCounty HospitalVeszprémHungary
  5. 5.Department of NeurologyUniversity of InnsbruckInnsbruckAustria
  6. 6.Department of Molecular NeuroscienceInstitute of NeurologyLondonUK
  7. 7.INSERM, UMR_S679 Neurologie and Thérapeutic ExpérimenteleAp-HP, Hôpital de la Pitié-SalpêtrièreParisFrance
  8. 8.Départment of GeneticsCytogenetics and Embryology, Salpetriere HospitalParisFrance
  9. 9.Department of NeurologyUniversity of NaplesNaplesItaly
  10. 10.Department of Neurology and Hertie-Institute for Clinical Brain ResearchUniversity of TübingenTubingenGermany
  11. 11.Department of Medical Genetics and Child DevelopmentUniversity of PécsPécsHungary
  12. 12.Department of Neurology, Hôpital ErasmeUniversité Libre de BruxellesBrusselsBelgium
  13. 13.Department of NeurologyUniversity of Duisburg-EssenEssenGermany
  14. 14.Department of NeurologyUniversity Medical Centre GroningenGroningenThe Netherlands
  15. 15.Donders Institute for Brain, Cognition and BehaviourCentre for NeuroscienceNijmegenThe Netherlands

Personalised recommendations