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Neurophysiological evidence for generalized sensory neuronopathy in cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome.
Muscle Nerve. 2015 Apr; 51(4):600-3.MN

Abstract

INTRODUCTION

Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) is a recently described multisystem ataxia defined by the presence of cerebellar ataxia, bilateral vestibulopathy, and a somatosensory deficit. The characteristic clinical sign is an abnormal visually enhanced vestibuloocular reflex. The somatosensory deficit contributes to a significant level of disability in CANVAS.

METHODS

This study was a neurophysiological investigation of 14 patients with CANVAS.

RESULTS

Findings revealed uniformly absent sensory nerve action potentials in all limbs, abnormal blink reflexes in 13 of 14 patients, and abnormal masseter reflexes in 6 of 11 patients. Tibial H-reflexes were absent in 11 of 14 patients. Somatosensory evoked potentials were abnormal in 10 of the 11 patients tested, and brainstem auditory evoked responses were abnormal in 3 of 8. Cutaneous silent period responses were abnormal in 7 of 14 patients.

CONCLUSIONS

We suggest that a sensory neuronopathy should be sought in cerebellar and/or vestibular ataxias, particularly where the degree of ataxia is out of proportion to the clinically identified cerebellar and/or vestibular dysfunction.

Authors+Show Affiliations

University of Melbourne, Royal Victorian Eye and Ear Hospital, Melbourne, Victoria, Australia.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

25130975

Citation

Szmulewicz, David J., et al. "Neurophysiological Evidence for Generalized Sensory Neuronopathy in Cerebellar Ataxia With Neuropathy and Bilateral Vestibular Areflexia Syndrome." Muscle & Nerve, vol. 51, no. 4, 2015, pp. 600-3.
Szmulewicz DJ, Seiderer L, Halmagyi GM, et al. Neurophysiological evidence for generalized sensory neuronopathy in cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome. Muscle Nerve. 2015;51(4):600-3.
Szmulewicz, D. J., Seiderer, L., Halmagyi, G. M., Storey, E., & Roberts, L. (2015). Neurophysiological evidence for generalized sensory neuronopathy in cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome. Muscle & Nerve, 51(4), 600-3. https://doi.org/10.1002/mus.24422
Szmulewicz DJ, et al. Neurophysiological Evidence for Generalized Sensory Neuronopathy in Cerebellar Ataxia With Neuropathy and Bilateral Vestibular Areflexia Syndrome. Muscle Nerve. 2015;51(4):600-3. PubMed PMID: 25130975.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Neurophysiological evidence for generalized sensory neuronopathy in cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome. AU - Szmulewicz,David J, AU - Seiderer,Linda, AU - Halmagyi,G Michael, AU - Storey,Elsdon, AU - Roberts,Leslie, Y1 - 2015/01/05/ PY - 2014/08/11/accepted PY - 2014/8/19/entrez PY - 2014/8/19/pubmed PY - 2015/5/21/medline KW - ataxia KW - cerebellar KW - ganglionopathy KW - neuronopathy KW - vestibular SP - 600 EP - 3 JF - Muscle & nerve JO - Muscle Nerve VL - 51 IS - 4 N2 - INTRODUCTION: Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) is a recently described multisystem ataxia defined by the presence of cerebellar ataxia, bilateral vestibulopathy, and a somatosensory deficit. The characteristic clinical sign is an abnormal visually enhanced vestibuloocular reflex. The somatosensory deficit contributes to a significant level of disability in CANVAS. METHODS: This study was a neurophysiological investigation of 14 patients with CANVAS. RESULTS: Findings revealed uniformly absent sensory nerve action potentials in all limbs, abnormal blink reflexes in 13 of 14 patients, and abnormal masseter reflexes in 6 of 11 patients. Tibial H-reflexes were absent in 11 of 14 patients. Somatosensory evoked potentials were abnormal in 10 of the 11 patients tested, and brainstem auditory evoked responses were abnormal in 3 of 8. Cutaneous silent period responses were abnormal in 7 of 14 patients. CONCLUSIONS: We suggest that a sensory neuronopathy should be sought in cerebellar and/or vestibular ataxias, particularly where the degree of ataxia is out of proportion to the clinically identified cerebellar and/or vestibular dysfunction. SN - 1097-4598 UR - https://wwww.unboundmedicine.com/medline/citation/25130975/Neurophysiological_evidence_for_generalized_sensory_neuronopathy_in_cerebellar_ataxia_with_neuropathy_and_bilateral_vestibular_areflexia_syndrome_ L2 - https://doi.org/10.1002/mus.24422 DB - PRIME DP - Unbound Medicine ER -