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May 31, 2011; 76 (22) Articles

Sensory neuropathy as part of the cerebellar ataxia neuropathy vestibular areflexia syndrome

D.J. Szmulewicz, J.A. Waterston, G.M. Halmagyi, S. Mossman, A.M. Chancellor, C.A. McLean, E. Storey
First published May 30, 2011, DOI: https://doi.org/10.1212/WNL.0b013e31821d746e
D.J. Szmulewicz
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J.A. Waterston
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G.M. Halmagyi
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S. Mossman
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A.M. Chancellor
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C.A. McLean
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E. Storey
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Citation
Sensory neuropathy as part of the cerebellar ataxia neuropathy vestibular areflexia syndrome
D.J. Szmulewicz, J.A. Waterston, G.M. Halmagyi, S. Mossman, A.M. Chancellor, C.A. McLean, E. Storey
Neurology May 2011, 76 (22) 1903-1910; DOI: 10.1212/WNL.0b013e31821d746e

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Abstract

Objective: The syndrome of cerebellar ataxia with bilateral vestibulopathy was delineated in 2004. Sensory neuropathy was mentioned in 3 of the 4 patients described. We aimed to characterize and estimate the frequency of neuropathy in this condition, and determine its typical MRI features.

Methods: Retrospective review of 18 subjects (including 4 from the original description) who met the criteria for bilateral vestibulopathy with cerebellar ataxia.

Results: The reported age at onset range was 39–71 years, and symptom duration was 3–38 years. The syndrome was identified in one sibling pair, suggesting that this may be a late-onset recessive disorder, although the other 16 cases were apparently sporadic. All 18 had sensory neuropathy with absent sensory nerve action potentials, although this was not apparent clinically in 2, and the presence of neuropathy was not a selection criterion. In 5, the loss of pinprick sensation was virtually global, mimicking a neuronopathy. However, findings in the other 11 with clinically manifest neuropathy suggested a length-dependent neuropathy. MRI scans showed cerebellar atrophy in 16, involving anterior and dorsal vermis, and hemispheric crus I, while 2 were normal. The inferior vermis and brainstem were spared.

Conclusions: Sensory neuropathy is an integral component of this syndrome. It may result in severe sensory loss, which contributes significantly to the disability. The MRI changes are nonspecific, but, coupled with loss of sensory nerve action potentials, may aid diagnosis. We propose a new name for the condition: cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS).

Footnotes

  • Supplemental data at www.neurology.org

  • 4WF
    4-wheeled frame
    CABV
    cerebellar ataxia with bilateral vestibulopathy
    CANVAS
    cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome
    CMAP
    compound muscle action potential
    FRDA
    Friedreich ataxia
    NCS
    nerve conduction study
    NCV
    nerve conduction velocity
    OKR
    optokinetic reflex
    SCA
    spinocerebellar ataxia
    SNAP
    sensory nerve action potential
    SPS
    single point stick
    VEMP
    vestibular evoked myogenic potential
    VOR
    vestibulo-ocular reflex
    VORS
    vestibulo-ocular reflex suppression
    VVOR
    visually enhanced vestibulo-ocular reflex.

  • Received December 24, 2010.
  • Accepted January 6, 2011.
  • Copyright © 2011 by AAN Enterprises, Inc.
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