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Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) - a case report and review of literature.
Neurol Neurochir Pol. 2014; 48(5):368-72.NN

Abstract

CANVAS (cerebellar ataxia with neuropathy and vestibular areflexia syndrome) is a rare neurological syndrome of unknown etiology. The main clinical features include bilateral vestibulopathy, cerebellar ataxia and sensory neuropathy. An abnormal visually enhanced vestibulo-ocular reflex is the hallmark of the disease. We present a case of 58-year-old male patient who has demonstrated gait disturbance, imbalance and paresthesia of feet for 2 years. On examination ataxia of gait, diminished knee and ankle reflexes, absence of plantar reflexes, fasciculations of thigh muscles, gaze-evoked downbeat nystagmus and abnormal visually enhanced vestibulo-ocular reflex were found. Brain magnetic resonance imaging revealed cerebellar atrophy. Vestibular function testing showed severely reduced horizontal nystagmus in response to bithermal caloric stimulation. Nerve conduction study revealed loss of upper and lower limb sensory nerve action potentials. The course of illness was progressive with ataxic gait and unsteadiness as the most disabling symptoms. We report 4-year follow-up of the patient since the beginning of the disease.

Authors+Show Affiliations

Department of Neurology, Medical University of Warsaw, Warsaw, Poland. Electronic address: mnkfigura@gmail.com.Department of Neurology, Medical University of Warsaw, Warsaw, Poland. Electronic address: malgorzata.gawel@wum.edu.pl.2nd Department of Clinical Radiology, Medical University of Warsaw, Warsaw, Poland. Electronic address: anna.kolasa@wp.pl.Department of Neurology, Medical University of Warsaw, Warsaw, Poland. Electronic address: piotr.janik@wum.edu.pl.

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

25440017

Citation

Figura, Monika, et al. "Cerebellar Ataxia With Neuropathy and Vestibular Areflexia Syndrome (CANVAS) - a Case Report and Review of Literature." Neurologia I Neurochirurgia Polska, vol. 48, no. 5, 2014, pp. 368-72.
Figura M, Gaweł M, Kolasa A, et al. Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) - a case report and review of literature. Neurol Neurochir Pol. 2014;48(5):368-72.
Figura, M., Gaweł, M., Kolasa, A., & Janik, P. (2014). Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) - a case report and review of literature. Neurologia I Neurochirurgia Polska, 48(5), 368-72. https://doi.org/10.1016/j.pjnns.2014.08.003
Figura M, et al. Cerebellar Ataxia With Neuropathy and Vestibular Areflexia Syndrome (CANVAS) - a Case Report and Review of Literature. Neurol Neurochir Pol. 2014;48(5):368-72. PubMed PMID: 25440017.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) - a case report and review of literature. AU - Figura,Monika, AU - Gaweł,Małgorzata, AU - Kolasa,Anna, AU - Janik,Piotr, Y1 - 2014/09/06/ PY - 2014/03/19/received PY - 2014/08/15/revised PY - 2014/08/27/accepted PY - 2014/12/3/entrez PY - 2014/12/3/pubmed PY - 2015/1/21/medline KW - Cerebellar ataxia KW - Cerebellar ataxia with neuropathy and vestibular areflexia syndrome KW - Sensory neuropathy KW - Vestibulopathy KW - Visually enhanced vestibulo-ocular reflex SP - 368 EP - 72 JF - Neurologia i neurochirurgia polska JO - Neurol Neurochir Pol VL - 48 IS - 5 N2 - CANVAS (cerebellar ataxia with neuropathy and vestibular areflexia syndrome) is a rare neurological syndrome of unknown etiology. The main clinical features include bilateral vestibulopathy, cerebellar ataxia and sensory neuropathy. An abnormal visually enhanced vestibulo-ocular reflex is the hallmark of the disease. We present a case of 58-year-old male patient who has demonstrated gait disturbance, imbalance and paresthesia of feet for 2 years. On examination ataxia of gait, diminished knee and ankle reflexes, absence of plantar reflexes, fasciculations of thigh muscles, gaze-evoked downbeat nystagmus and abnormal visually enhanced vestibulo-ocular reflex were found. Brain magnetic resonance imaging revealed cerebellar atrophy. Vestibular function testing showed severely reduced horizontal nystagmus in response to bithermal caloric stimulation. Nerve conduction study revealed loss of upper and lower limb sensory nerve action potentials. The course of illness was progressive with ataxic gait and unsteadiness as the most disabling symptoms. We report 4-year follow-up of the patient since the beginning of the disease. SN - 0028-3843 UR - https://wwww.unboundmedicine.com/medline/citation/25440017/Cerebellar_ataxia_with_neuropathy_and_vestibular_areflexia_syndrome__CANVAS____a_case_report_and_review_of_literature_ DB - PRIME DP - Unbound Medicine ER -