Skip to main content

Advertisement

Springer Nature Link
Log in

High Width Variability during Spiral Drawing: Further Evidence of Cerebellar Dysfunction in Essential Tremor

  • Original Paper
  • Published:
The Cerebellum Aims and scope Submit manuscript

Abstract

Essential tremor (ET) is among the most prevalent neurological diseases, yet the location of the primary disease substrate continues to be a matter of debate. The presence of intention tremor and mild gait ataxia suggests an underlying abnormality of the cerebellum and/or cerebellar pathways. Uncovering additional signs of cerebellar dysfunction would further substantiate the proposition that ET is a disease of the cerebellar system. We evaluated 145 ET cases and 34 normal controls clinically and by computerized spiral analysis. Spiral analysis is a program that objectively characterizes kinematic and physiologic features of hand-drawn spirals using specific calculated spiral indices that correlate with spiral shape and motor execution. We used the spiral width variability index (SWVI), a measure of loop-to-loop spiral width variation with the influence of tremor removed, as a metric of drawing ataxia. The SWVI was higher in cases than controls (0.91 ± 1.94, median = 0.46 vs. 0.40 ± 0.29, median = 0.30, p < 0.001). Cases with higher SWVI also had greater intention tremor during the finger–nose–finger maneuver, r = 0.27, p = 0.001), and cases with intention tremor of the head had the highest SWVI (1.57 ± 3.44, median = 0.51, p < 0.001). There was a modest association between SWVI and number of missteps during tandem gait (r = 0.16, p = 0.06). The primary anatomical substrate for ET continues to be a matter of speculation, yet these and other clinical data lend support to the notion that there is an underlying abnormality of the cerebellum and/or its pathways.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

Explore related subjects

Discover the latest articles and news from researchers in related subjects, suggested using machine learning.

References

  1. Louis ED, Ferreira JJ. How common is the most common adult movement disorder? Update on the worldwide prevalence of essential tremor. Mov Disord. 2010;25:534–41.

    Article  PubMed  Google Scholar 

  2. Benito-Leon J, Bermejo-Pareja F, Morales JM, Vega S, Molina JA. Prevalence of essential tremor in three elderly populations of Central Spain. Mov Disord. 2003;18:389–94.

    Article  PubMed  Google Scholar 

  3. Louis ED, Frucht SJ, Rios E. Intention tremor in essential tremor: prevalence and association with disease duration. Mov Disord. 2009;24:626–7.

    Article  PubMed  Google Scholar 

  4. Leegwater-Kim J, Louis ED, Pullman SL, Floyd AG, Borden S, Moskowitz CB, et al. Intention tremor of the head in patients with essential tremor. Mov Disord. 2006;21:2001–5.

    Article  PubMed  Google Scholar 

  5. Stolze H, Petersen G, Raethjen J, Wenzelburger R, Deuschl G. The gait disorder of advanced essential tremor. Brain. 2001;124:2278–86.

    Article  PubMed  CAS  Google Scholar 

  6. Louis ED, Rios E, Rao AK. Tandem gait performance in essential tremor: clinical correlates and association with midline tremors. Mov Disord. 2010;25:1633–8.

    Article  PubMed  Google Scholar 

  7. Rao AK, Gillman A, Louis ED. Quantitative gait analysis in essential tremor reveals impairments that are maintained into advanced age. Gait Posture. 2011;34:65–70.

    Article  PubMed  Google Scholar 

  8. Helmchen C, Hagenow A, Miesner J, Sprenger A, Rambold H, Wenzelburger R, et al. Eye movement abnormalities in essential tremor may indicate cerebellar dysfunction. Brain. 2003;126:1319–32.

    Article  PubMed  CAS  Google Scholar 

  9. Trillenberg P, Fuhrer J, Sprenger A, Hagenow A, Kompf D, Wenzelburger R, et al. Eye–hand coordination in essential tremor. Mov Disord. 2006;21:373–9.

    Article  PubMed  Google Scholar 

  10. Farkas Z, Szirmai I, Kamondi A. Impaired rhythm generation in essential tremor. Mov Disord. 2006;21:1196–9.

    Article  PubMed  Google Scholar 

  11. Bares M, Lungu OV, Husarova I, Gescheidt T. Predictive motor timing performance dissociates between early diseases of the cerebellum and Parkinson's disease. Cerebellum. 2010;9:124–35.

    Article  PubMed  Google Scholar 

  12. Kronenbuerger M, Gerwig M, Brol B, Block F, Timmann D. Eyeblink conditioning is impaired in subjects with essential tremor. Brain. 2007;130:1538–51.

    Article  PubMed  Google Scholar 

  13. Quattrone A, Cerasa A, Messina D, Nicoletti G, Hagberg GE, Lemieux L, et al. Essential head tremor is associated with cerebellar vermis atrophy: a volumetric and voxel-based morphometry MR imaging study. AJNR Am J Neuroradiol. 2008;29:1692–7.

    Article  PubMed  CAS  Google Scholar 

  14. Nicoletti G, Manners D, Novellino F, Condino F, Malucelli E, Barbiroli B, et al. Diffusion tensor MRI changes in cerebellar structures of patients with familial essential tremor. Neurology. 2010;74:988–94.

    Article  PubMed  CAS  Google Scholar 

  15. Louis ED, Shungu DC, Chan S, Mao X, Jurewicz EC, Watner D. Metabolic abnormality in the cerebellum in patients with essential tremor: a proton magnetic resonance spectroscopic imaging study. Neurosci Lett. 2002;333:17–20.

    Article  PubMed  CAS  Google Scholar 

  16. Benito-Leon J, Alvarez-Linera J, Hernandez-Tamames JA, Alonso-Navarro H, Jimenez-Jimenez FJ, Louis ED. Brain structural changes in essential tremor: voxel-based morphometry at 3-tesla. J Neurol Sci. 2009;287:138–42.

    Article  PubMed  Google Scholar 

  17. Cerasa A, Messina D, Nicoletti G, Novellino F, Lanza P, Condino F, et al. Cerebellar atrophy in essential tremor using an automated segmentation method. AJNR Am J Neuroradiol. 2009;30:1240–3.

    Article  PubMed  CAS  Google Scholar 

  18. Passamonti L, Novellino F, Cerasa A, Chiriaco C, Rocca F, Matina MS, et al. Altered cortical-cerebellar circuits during verbal working memory in essential tremor. Brain. 2011;134:2274–86.

    Article  PubMed  Google Scholar 

  19. Kuo SH, Erickson-Davis C, Gillman A, Faust PL, Vonsattel JP, Louis ED. Increased number of heterotopic Purkinje cells in essential tremor. J Neurol Neurosurg Psychiatry. 2011;82:1038–40.

    Article  PubMed  Google Scholar 

  20. Louis ED. Essential tremor: evolving clinicopathological concepts in an era of intensive post-mortem enquiry. Lancet Neurol. 2010;9:613–22.

    Article  PubMed  Google Scholar 

  21. Kuo SH, Faust PL, Vonsattel JP, Ma K, Louis ED. Parallel fiber counts and parallel fiber integrated density are similar in essential tremor cases and controls. Acta Neuropathol. 2011;121:287–9.

    Article  PubMed  Google Scholar 

  22. Erickson-Davis CR, Faust PL, Vonsattel JP, Gupta S, Honig LS, Louis ED. "Hairy baskets" associated with degenerative Purkinje cell changes in essential tremor. J Neuropathol Exp Neurol. 2010;69:262–71.

    Article  PubMed  Google Scholar 

  23. Louis ED, Faust PL, Ma KJ, Yu M, Cortes E, Vonsattel JP. Torpedoes in the cerebellar vermis in essential tremor cases vs controls. Cerebellum. 2011;4:812–9.

    Article  Google Scholar 

  24. Pullman SL. Spiral analysis: a new technique for measuring tremor with a digitizing tablet. Mov Disord. 1998;13 Suppl 3:8589.

    Google Scholar 

  25. Louis ED, Yu Q, Floyd AG, Moskowitz C, Pullman SL. Axis is a feature of handwritten spirals in essential tremor. Mov Disord. 2006;21:1294–5.

    Article  PubMed  Google Scholar 

  26. Hsu AW, Piboolnurak PA, Floyd AG, Yu QP, Wraith JE, Patterson MC, et al. Spiral analysis in Niemann–Pick disease type C. Mov Disord. 2009;24:1984–90.

    Article  PubMed  Google Scholar 

  27. Louis ED, Faust PL, Vonsattel JP, Honig LS, Rajput A, Robinson CA, et al. Neuropathological changes in essential tremor: 33 cases compared with 21 controls. Brain. 2007;130:3297–307.

    Article  PubMed  Google Scholar 

  28. Louis ED, Borden S, Moskowitz CB. Essential tremor centralized brain repository: diagnostic validity and clinical characteristics of a highly selected group of essential tremor cases. Mov Disord. 2005;20:1361–5.

    Article  PubMed  Google Scholar 

  29. Louis ED, Rios E, Henchcliffe C. Mirror movements in patients with essential tremor. Mov Disord. 2009;24:2211–7.

    Article  PubMed  Google Scholar 

  30. Fahn S, Elton R, Members of the UPDRS Development Committee in Fahn S, Marsden C, Goldtein M, Calne DB (ed) (1987) Recent developments in Parkinson's disease. Florham Park, NJ, Macmillan Health Care Information. pp 153–63.

  31. Longstaff MG, Heath RA. Spiral drawing performance as an indicator of fine motor function in people with multiple sclerosis. Hum Mov Sci. 2006;25:474–91.

    Article  PubMed  CAS  Google Scholar 

  32. Louis ED, Ottman R, Ford B, Pullman S, Martinez M, Fahn S, et al. The Washington Heights-Inwood Genetic Study of essential tremor: methodologic issues in essential tremor research. Neuroepidemiology. 1997;16:124–33.

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

R01 NS042859 from the National Institutes of Health (Bethesda, MD, USA; EDL) and support from the Parkinson Disease Foundation (New York, NY, USA; CWH, SLP).

Author information

Authors and Affiliations

  1. GH Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, NY, USA

    Elan D. Louis & Arthur Gillman

  2. Department of Neurology, College of Physicians New York, New York, NY, USA

    Elan D. Louis

  3. Taub Institute for Research on Alzheimer’s Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, NY, USA

    Elan D. Louis

  4. Department of Epidemiology, Mailman School of Public Health, Columbia University, New York, NY, USA

    Elan D. Louis

  5. Clinical Motor Physiology Laboratory, Department of Neurology, College of Physicians, New York, NY, USA

    Sarah Boschung, Christopher W. Hess, Qiping Yu & Seth L. Pullman

  6. Unit 198, Neurological Institute, 710 West 168th Street, New York, NY, 10032, USA

    Elan D. Louis

Authors
  1. Elan D. Louis
    View author publications

    You can also search for this author inPubMed Google Scholar

  2. Arthur Gillman
    View author publications

    You can also search for this author inPubMed Google Scholar

  3. Sarah Boschung
    View author publications

    You can also search for this author inPubMed Google Scholar

  4. Christopher W. Hess
    View author publications

    You can also search for this author inPubMed Google Scholar

  5. Qiping Yu
    View author publications

    You can also search for this author inPubMed Google Scholar

  6. Seth L. Pullman
    View author publications

    You can also search for this author inPubMed Google Scholar

Corresponding author

Correspondence to Elan D. Louis.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Louis, E.D., Gillman, A., Boschung, S. et al. High Width Variability during Spiral Drawing: Further Evidence of Cerebellar Dysfunction in Essential Tremor. Cerebellum 11, 872–879 (2012). https://doi.org/10.1007/s12311-011-0352-4

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12311-011-0352-4

Keywords