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VEMPs and Dysautonomia Assessment in Definite Cerebellar Ataxia, Neuropathy, Vestibular Areflexia Syndrome (CANVAS): a Case Series Study

  • David Moreno-Ajona
  • Laura Álvarez-Gómez
  • Raquel Manrique-Huarte
  • Estefanía Rivas
  • Eduardo Martínez-Vila
  • Nicolás Pérez-FernándezEmail author
Short Report
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Abstract

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a recently described slowly progressive ataxia with severe imbalance due to the compromise of three of the four sensory inputs for balance, leaving only vision unaffected. Bilateral vestibulopathy is present but saccular and utricular function, measured by vestibular evoked myogenic potentials (VEMPs), has not been widely studied in these patients. Dysautonomia has been reported but is not among the diagnostic criteria. We performed a database analysis to identify patients evaluated between 2003 and 2019 with probable diagnosis of CANVAS by using key words “bilateral vestibulopathy and/or cerebellar ataxia and/or sensory polyneuropathy.” Five out of 842 met all conditions. Patients underwent neurological/neurootological exam, brain MRI, visually enhanced vestibulo-ocular reflex (VVOR) exam by high-speed video-oculography using video-Head Impulse Test (vHIT), VEMPs, neurophysiological studies, and genetic tests to exclude other causes of ataxia. Dysautonomia was addressed by the standardized survey of autonomic symptoms. All patients had clinically definite CANVAS as brain MRI showed vermal cerebellar atrophy, neurophysiological studies showed a sensory neuronopathy pattern (absent sensory action potentials), VVOR was abnormal bilaterally, and genetic tests ruled out other causes of ataxia including SCA 3 and Friedreich ataxia. Patients had at least 3 dysautonomic symptoms, including xerostomia/xerophthalmia (5/5). VEMP results varied among patients, ranging from normal to completely abnormal. We found inconsistent results with VEMPs. The utilization of VEMPs in more CANVAS cases will determine its utility in this syndrome. Dysautonomia may be included in the diagnostic criteria.

Keywords

Cerebellar ataxia Bilateral vestibulopathy Polyneuropathy Dysautonomia 

Notes

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Departamento de NeurologíaClínica Universidad de NavarraPamplonaSpain
  2. 2.Departamento de OtorrinolaringologíaClínica Universidad de NavarraPamplonaSpain
  3. 3.Departamento de NeurofisiologíaClínica Universidad de NavarraPamplonaSpain
  4. 4.Departamento de OtorrinolaringologíaClínica Universidad de NavarraMadridSpain

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