Skip to main content

Advertisement

Log in

What Is Behind Cerebellar Vertigo and Dizziness?

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

Abstract

The differential diagnosis of vertigo or dizziness as a result of cerebellar disorders can be difficult as many patients with a cerebellar pathology do not present with the full spectrum of cerebellar signs. The main goal of this study was to describe the typical clinical features of these patients with vertigo or dizziness of a cerebellar origin. We reviewed the medical records of 5400 patients with vertigo and dizziness from our tertiary outpatient clinic for vertigo and balance disorders. In 459 the diagnosis of “cerebellar vertigo or dizziness” was made; 90 patients were excluded from further analysis due to evident structural changes in MRI. Of the remaining 369 patients (67.0 ± 15.1, 54% female, symptom duration until diagnosis 5.5 ± 6.9 years), 81% suffered from persistent vertigo or dizziness, 31% from attacks of vertigo and dizziness and 21% from both. Neuro-ophthalmologically, 95% had saccadic smooth pursuit, 80% gaze-holding deficits, 64% a pathological fixation suppression of the VOR, 24% central fixation nystagmus (in 64% of these cases downbeat nystagmus (DBN)), 23% rebound nystagmus, and an ocular misalignment in 84% in near view and 50% in distance view. Eleven percent had isolated mild to moderate cerebellar ocular motor disturbances without any other typical cerebellar signs. The most common diagnoses were sporadic adult-onset degenerative ataxia in 26%; idiopathic DBN syndrome in 20%; cerebellar ataxia, neuropathy, and vestibular areflexia syndrome in 10%; episodic ataxia type 2 in 7%; and multiple system atrophy cerebellar type in 6%. In posturography, a typical cerebellar 3-Hz sway was found in 16%. The diagnostic key to patients with cerebellar vertigo or dizziness is a careful examination of eye movements since practically all of them have cerebellar ocular disturbances.

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

Access this article

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

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Corrales CE, Bhattacharyya N. Dizziness and death: an imbalance in mortality. Laryngoscope. 2016;126(9):2134–6.

    Article  PubMed  Google Scholar 

  2. Brandt T, Dieterich M, Strupp M. Vertigo and dizziness - common complaints, Vol. 2. Springer; 2013.

  3. Kim JS, Lee H. Vertigo due to posterior circulation stroke. Semin Neurol. 2013;33(3):179–84.

    Article  PubMed  Google Scholar 

  4. Lee H. Neuro-otological aspects of cerebellar stroke syndrome. J Clin Neurol. 2009;5(2):65–73.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Grimaldi G, Manto M. Topography of cerebellar deficits in humans. Cerebellum. 2012;11(2):336–51.

    Article  PubMed  Google Scholar 

  6. Bodranghien F, Bastian A, Casali C, Hallett M, Louis ED, Manto M, et al. Consensus paper: revisiting the symptoms and signs of cerebellar syndrome. Cerebellum. 2016;15(3):369–91.

    Article  PubMed  PubMed Central  Google Scholar 

  7. Leigh RJ, Zee DS, editors. The Neurology of Eye Movements. 5th ed. Oxford, New York: Oxford University Press; 2015.

    Google Scholar 

  8. Diener HC, Dichgans J, Bacher M, Gompf B. Quantification of postural sway in normals and patients with cerebellar diseases. Electroencephalogr Clin Neurophysiol. 1984;57(2):134–42.

    Article  CAS  PubMed  Google Scholar 

  9. Baloh RW, Konrad HR, Honrubia V. Vestibulo-ocular function in patients with cerebellar atrophy. Neurology. 1975;25(2):160–8.

    Article  CAS  PubMed  Google Scholar 

  10. Thurston SE, Leigh RJ, Abel LA, Dell'Osso LF. Hyperactive vestibulo-ocular reflex in cerebellar degeneration: pathogenesis and treatment. Neurology. 1987;37(1):53–7.

    Article  CAS  PubMed  Google Scholar 

  11. Klockgether T. Sporadic adult-onset ataxia. Handb Clin Neurol. 2018;155:217–25.

    Article  PubMed  Google Scholar 

  12. Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ, et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008;71(9):670–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Szmulewicz DJ, Roberts L, McLean CA, MacDougall HG, Halmagyi GM, Storey E. Proposed diagnostic criteria for cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS). Neurol Clin Pract. 2016;6(1):61–8.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Hufner K, et al. Esophoria or esotropia in adulthood: a sign of cerebellar dysfunction? J Neurol. 2015;262(3):585–92.

    Article  PubMed  Google Scholar 

  15. Schneider E, Villgrattner T, Vockeroth J, Bartl K, Kohlbecher S, Bardins S, et al. EyeSeeCam: an eye movement-driven head camera for the examination of natural visual exploration. Ann N Y Acad Sci. 2009;1164:461–7.

    Article  PubMed  Google Scholar 

  16. Bremova-Ertl T, et al. Oculomotor and vestibular findings in Gaucher disease type 3 and their correlation with neurological findings. Front Neurol. 2017;8:711.

    Article  PubMed  Google Scholar 

  17. Strupp M, Kim JS, Murofushi T, Straumann D, Jen JC, Rosengren SM, et al. Bilateral vestibulopathy: diagnostic criteria consensus document of the classification Committee of the Barany Society. J Vestib Res. 2017;27(4):177–89.

    Article  PubMed  Google Scholar 

  18. Krafczyk S, Tietze S, Swoboda W, Valkovič P, Brandt T. Artificial neural network: a new diagnostic posturographic tool for disorders of stance. Clin Neurophysiol. 2006;117(8):1692–8.

    Article  PubMed  Google Scholar 

  19. Subramony SH. SARA--a new clinical scale for the assessment and rating of ataxia. Nat Clin Pract Neurol. 2007;3(3):136–7.

    Article  PubMed  Google Scholar 

  20. R Core Team. R: a language and environment for statistical computing. Vienna: R Foundation for Statistical Computing; 2018. https://www.R-project.org/.

  21. Claassen J, Feil K, Bardins S, Teufel J, Spiegel R, Kalla R, et al. Dalfampridine in patients with downbeat nystagmus--an observational study. J Neurol. 2013;260(8):1992–6.

    Article  CAS  PubMed  Google Scholar 

  22. Claassen J, Spiegel R, Kalla R, Faldon M, Kennard C, Danchaivijitr C, et al. A randomised double-blind, cross-over trial of 4-aminopyridine for downbeat nystagmus--effects on slowphase eye velocity, postural stability, locomotion and symptoms. J Neurol Neurosurg Psychiatry. 2013;84(12):1392–9.

    Article  PubMed  Google Scholar 

  23. Claassen J, Teufel J, Kalla R, Spiegel R, Strupp M. Effects of dalfampridine on attacks in patients with episodic ataxia type 2: an observational study. J Neurol. 2013;260(2):668–9.

    Article  PubMed  Google Scholar 

  24. Strupp M, Kalla R, Claassen J, Adrion C, Mansmann U, Klopstock T, et al. A randomized trial of 4-aminopyridine in EA2 and related familial episodic ataxias. Neurology. 2011;77(3):269–75.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Strupp M, Kalla R, Dichgans M, Freilinger T, Glasauer S, Brandt T. Treatment of episodic ataxia type 2 with the potassium channel blocker 4-aminopyridine. Neurology. 2004;62(9):1623–5.

    Article  CAS  PubMed  Google Scholar 

  26. Strupp M, Schuler O, Krafczyk S, Jahn K, Schautzer F, Buttner U, et al. Treatment of downbeat nystagmus with 3,4-diaminopyridine: a placebo-controlled study. Neurology. 2003;61(2):165–70.

    Article  CAS  PubMed  Google Scholar 

  27. Strupp M, Teufel J, Habs M, Feuerecker R, Muth C, van de Warrenburg BP, et al. Effects of acetyl-DL-leucine in patients with cerebellar ataxia: a case series. J Neurol. 2013;260(10):2556–61.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Li X, Wang Y, Wang Z, Xu Y, Zheng W. 3-Hz postural tremor in multiple system atrophy cerebellar type (MSA-C)-a static posturography study. Neurol Sci. 2018;39(1):71–7.

    Article  CAS  PubMed  Google Scholar 

  29. Kremmyda O, et al. False-positive head-impulse test in cerebellar ataxia. Front Neurol. 2012;3:162.

    Article  PubMed  PubMed Central  Google Scholar 

  30. Kirchner H, Kremmyda O, Hüfner K, Stephan T, Zingler V, Brandt T, et al. Clinical, electrophysiological, and MRI findings in patients with cerebellar ataxia and a bilaterally pathological head-impulse test. Ann N Y Acad Sci. 2011;1233:127–38.

    Article  PubMed  Google Scholar 

  31. Bronstein AM, Hood JD. Cervical nystagmus due to loss of cerebellar inhibition on the cervico-ocular reflex: a case report. J Neurol Neurosurg Psychiatry. 1985;48(2):128–31.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Yu-Wai-Man P, Gorman G, Bateman DE, Leigh RJ, Chinnery PF. Vertigo and vestibular abnormalities in spinocerebellar ataxia type 6. J Neurol. 2009;256(1):78–82.

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

We thank Katie Göttlinger for copy-editing the manuscript. We thank the team of the neuro-orthoptists for the neuro-ophthalmological examination of the patients.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Katharina Feil.

Ethics declarations

The study was approved by the ethics committee of the Ludwig-Maximilians University Munich and was performed in accordance with the ethical standards according to the 1964 Declaration of Helsinki and its later amendments.

Competing Interests

K. Feil reports no disclosures.

R. Strobl reports no disclosures.

A. Schindler reports no disclosures.

S: Krafczyk reports no disclosures.

N. Goldschagg received honoraria for lecturing from Actelion.

C. Frenzel reports no disclosures.

M. Glaser reports no disclosures.

F. Schöberl reports no disclosures.

A. Zwergal reports no disclosures.

M. Strupp is Joint Chief Editor of the Journal of Neurology, Editor in Chief of Frontiers of Neuro-otology, and Section Editor of F1000. He has received speaker’s honoraria from Abbott, Actelion, Auris Medical, Biogen, Eisai, Grünenthal, GSK, Henning Pharma, Interacoustics, MSD, Otometrics, Pierre-Fabre, TEVA, and UCB. He is a shareholder in IntraBio. He acts as a consultant for Abbott, Actelion, AurisMedical, Heel, IntraBio, and Sensorion.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Electronic Supplementary Material

ESM 1

(DOCX 43 kb)

ESM 2

(JPG 30 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Feil, K., Strobl, R., Schindler, A. et al. What Is Behind Cerebellar Vertigo and Dizziness?. Cerebellum 18, 320–332 (2019). https://doi.org/10.1007/s12311-018-0992-8

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12311-018-0992-8

Keywords

Navigation