Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) - a case report and review of literature
- PMID: 25440017
- DOI: 10.1016/j.pjnns.2014.08.003
Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) - a case report and review of literature
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.
Keywords: Cerebellar ataxia; Cerebellar ataxia with neuropathy and vestibular areflexia syndrome; Sensory neuropathy; Vestibulopathy; Visually enhanced vestibulo-ocular reflex.
Copyright © 2014 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.
Similar articles
-
[Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS): a case report].Rinsho Shinkeigaku. 2019 Jan 30;59(1):27-32. doi: 10.5692/clinicalneurol.cn-001209. Epub 2018 Dec 29. Rinsho Shinkeigaku. 2019. PMID: 30606994 Japanese.
-
Cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) with chronic cough and preserved muscle stretch reflexes: evidence for selective sparing of afferent Ia fibres.J Neurol. 2018 Jun;265(6):1454-1462. doi: 10.1007/s00415-018-8872-1. Epub 2018 Apr 25. J Neurol. 2018. PMID: 29696497
-
Neurophysiological evidence for generalized sensory neuronopathy in cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome.Muscle Nerve. 2015 Apr;51(4):600-3. doi: 10.1002/mus.24422. Epub 2015 Jan 5. Muscle Nerve. 2015. PMID: 25130975
-
Cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS): a review of the clinical features and video-oculographic diagnosis.Ann N Y Acad Sci. 2011 Sep;1233:139-47. doi: 10.1111/j.1749-6632.2011.06158.x. Ann N Y Acad Sci. 2011. PMID: 21950986 Review.
-
CANVAS an update: clinical presentation, investigation and management.J Vestib Res. 2014;24(5-6):465-74. doi: 10.3233/VES-140536. J Vestib Res. 2014. PMID: 25564090 Review.
Publication types
MeSH terms
LinkOut - more resources
Other Literature Sources
Medical