open access

Vol 48, No 5 (2014)
Case reports
Submitted: 2014-03-19
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Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) – A case report and review of literature

Monika Figura, Małgorzata Gaweł, Anna Kolasa, Piotr Janik
DOI: 10.1016/j.pjnns.2014.08.003
·
Neurol Neurochir Pol 2014;48(5):368-372.

open access

Vol 48, No 5 (2014)
Case reports
Submitted: 2014-03-19

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.

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.

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Keywords

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome, Cerebellar ataxia, Sensory neuropathy, Vestibulopathy, Visually enhanced vestibulo-ocular reflex

About this article
Title

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) – A case report and review of literature

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 48, No 5 (2014)

Pages

368-372

DOI

10.1016/j.pjnns.2014.08.003

Bibliographic record

Neurol Neurochir Pol 2014;48(5):368-372.

Keywords

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome
Cerebellar ataxia
Sensory neuropathy
Vestibulopathy
Visually enhanced vestibulo-ocular reflex

Authors

Monika Figura
Małgorzata Gaweł
Anna Kolasa
Piotr Janik

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