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Peripheral nerves are pathologically small in cerebellar ataxia neuropathy vestibular areflexia syndrome: a controlled ultrasound study.
Eur J Neurol. 2018 04; 25(4):659-665.EJ

Abstract

BACKGROUND AND PURPOSE

Sensory neuronopathy is a cardinal feature of cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS). Having observed that two patients with CANVAS had small median and ulnar nerves on ultrasound, we set out to examine this finding systematically in a cohort of patients with CANVAS, and compare them with both healthy controls and a cohort of patients with axonal neuropathy. We have previously reported preliminary findings in seven of these patients with CANVAS and seven healthy controls.

METHODS

We compared the ultrasound cross-sectional area of median, ulnar, sural and tibial nerves of 14 patients with CANVAS with 14 healthy controls and 14 age- and gender-matched patients with acquired primarily axonal neuropathy. We also compared the individual nerve cross-sectional areas of patients with CANVAS and neuropathy with the reference values of our laboratory control population.

RESULTS

The nerve cross-sectional area of patients with CANVAS was smaller than that of both the healthy controls and the neuropathy controls, with highly significant differences at most sites (P < 0.001). Conversely, the nerve cross-sectional areas in the upper limb were larger in neuropathy controls than healthy controls (P < 0.05). On individual analysis, the ultrasound abnormality was sufficiently characteristic to be detected in all but one patient with CANVAS.

DISCUSSION

Small nerves in CANVAS probably reflect nerve thinning from loss of axons due to ganglion cell loss. This is distinct from the ultrasound findings in axonal neuropathy, in which nerve size was either normal or enlarged. Our findings indicate a diagnostic role for ultrasound in CANVAS sensory neuronopathy and in differentiating neuronopathy from neuropathy.

Authors+Show Affiliations

Department of Neurology and Clinical Neurophysiology, Bay of Plenty District Health Board, Tauranga.Auckland District Health Board, Auckland.Capital and Coast District Health Board, Wellington.Auckland District Health Board, Auckland.Department of Neurology and Clinical Neurophysiology, Bay of Plenty District Health Board, Tauranga.Capital and Coast District Health Board, Wellington.Department of Endocrinology, Bay of Plenty District Health Board, Tauranga.Christchurch Hospital, Christchurch.Auckland District Health Board, Auckland. Department of Medicine, University of Auckland, Auckland, New Zealand.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

29316033

Citation

Pelosi, L, et al. "Peripheral Nerves Are Pathologically Small in Cerebellar Ataxia Neuropathy Vestibular Areflexia Syndrome: a Controlled Ultrasound Study." European Journal of Neurology, vol. 25, no. 4, 2018, pp. 659-665.
Pelosi L, Mulroy E, Leadbetter R, et al. Peripheral nerves are pathologically small in cerebellar ataxia neuropathy vestibular areflexia syndrome: a controlled ultrasound study. Eur J Neurol. 2018;25(4):659-665.
Pelosi, L., Mulroy, E., Leadbetter, R., Kilfoyle, D., Chancellor, A. M., Mossman, S., Wing, L., Wu, T. Y., & Roxburgh, R. H. (2018). Peripheral nerves are pathologically small in cerebellar ataxia neuropathy vestibular areflexia syndrome: a controlled ultrasound study. European Journal of Neurology, 25(4), 659-665. https://doi.org/10.1111/ene.13563
Pelosi L, et al. Peripheral Nerves Are Pathologically Small in Cerebellar Ataxia Neuropathy Vestibular Areflexia Syndrome: a Controlled Ultrasound Study. Eur J Neurol. 2018;25(4):659-665. PubMed PMID: 29316033.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Peripheral nerves are pathologically small in cerebellar ataxia neuropathy vestibular areflexia syndrome: a controlled ultrasound study. AU - Pelosi,L, AU - Mulroy,E, AU - Leadbetter,R, AU - Kilfoyle,D, AU - Chancellor,A M, AU - Mossman,S, AU - Wing,L, AU - Wu,T Y, AU - Roxburgh,R H, Y1 - 2018/02/26/ PY - 2017/09/11/received PY - 2017/12/27/accepted PY - 2018/1/10/pubmed PY - 2019/2/5/medline PY - 2018/1/10/entrez KW - axonal neuropathy KW - cerebellar ataxia neuropathy vestibular areflexia syndrome KW - cross-sectional area KW - nerve ultrasound KW - neuropathy KW - sensory neuronopathy SP - 659 EP - 665 JF - European journal of neurology JO - Eur J Neurol VL - 25 IS - 4 N2 - BACKGROUND AND PURPOSE: Sensory neuronopathy is a cardinal feature of cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS). Having observed that two patients with CANVAS had small median and ulnar nerves on ultrasound, we set out to examine this finding systematically in a cohort of patients with CANVAS, and compare them with both healthy controls and a cohort of patients with axonal neuropathy. We have previously reported preliminary findings in seven of these patients with CANVAS and seven healthy controls. METHODS: We compared the ultrasound cross-sectional area of median, ulnar, sural and tibial nerves of 14 patients with CANVAS with 14 healthy controls and 14 age- and gender-matched patients with acquired primarily axonal neuropathy. We also compared the individual nerve cross-sectional areas of patients with CANVAS and neuropathy with the reference values of our laboratory control population. RESULTS: The nerve cross-sectional area of patients with CANVAS was smaller than that of both the healthy controls and the neuropathy controls, with highly significant differences at most sites (P < 0.001). Conversely, the nerve cross-sectional areas in the upper limb were larger in neuropathy controls than healthy controls (P < 0.05). On individual analysis, the ultrasound abnormality was sufficiently characteristic to be detected in all but one patient with CANVAS. DISCUSSION: Small nerves in CANVAS probably reflect nerve thinning from loss of axons due to ganglion cell loss. This is distinct from the ultrasound findings in axonal neuropathy, in which nerve size was either normal or enlarged. Our findings indicate a diagnostic role for ultrasound in CANVAS sensory neuronopathy and in differentiating neuronopathy from neuropathy. SN - 1468-1331 UR - https://wwww.unboundmedicine.com/medline/citation/29316033/Peripheral_nerves_are_pathologically_small_in_cerebellar_ataxia_neuropathy_vestibular_areflexia_syndrome:_a_controlled_ultrasound_study_ DB - PRIME DP - Unbound Medicine ER -