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Peripheral nerves are pathologically small in cerebellar ataxia neuropathy vestibular areflexia syndrome: a controlled ultrasound study.

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Title: Peripheral nerves are pathologically small in cerebellar ataxia neuropathy vestibular areflexia syndrome: a controlled ultrasound study.
Authors: Pelosi, L., Mulroy, E., Leadbetter, R., Kilfoyle, D., Chancellor, A. M., Mossman, S., Wing, L., Wu, T. Y., Roxburgh, R. H.
Source: European Journal of Neurology. Apr2018, Vol. 25 Issue 4, p659-665. 7p. 1 Diagram, 2 Charts, 1 Graph.
Subjects: Cerebellar ataxia, Neuropathy, Nerves, Ultrasonic imaging, Vestibular stimulation
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. [ABSTRACT FROM AUTHOR]
Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
Database: Psychology and Behavioral Sciences Collection
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  Data: Peripheral nerves are pathologically small in cerebellar ataxia neuropathy vestibular areflexia syndrome: a controlled ultrasound study.
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  Data: &lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Pelosi%2C+L%2E%22&quot;&gt;Pelosi, L.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Mulroy%2C+E%2E%22&quot;&gt;Mulroy, E.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Leadbetter%2C+R%2E%22&quot;&gt;Leadbetter, R.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Kilfoyle%2C+D%2E%22&quot;&gt;Kilfoyle, D.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Chancellor%2C+A%2E+M%2E%22&quot;&gt;Chancellor, A. M.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Mossman%2C+S%2E%22&quot;&gt;Mossman, S.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Wing%2C+L%2E%22&quot;&gt;Wing, L.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Wu%2C+T%2E+Y%2E%22&quot;&gt;Wu, T. Y.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Roxburgh%2C+R%2E+H%2E%22&quot;&gt;Roxburgh, R. H.&lt;/searchLink&gt;
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  Data: &lt;searchLink fieldCode=&quot;JN&quot; term=&quot;%22European+Journal+of+Neurology%22&quot;&gt;European Journal of Neurology&lt;/searchLink&gt;. Apr2018, Vol. 25 Issue 4, p659-665. 7p. 1 Diagram, 2 Charts, 1 Graph.
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  Data: &lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Cerebellar+ataxia%22&quot;&gt;Cerebellar ataxia&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Neuropathy%22&quot;&gt;Neuropathy&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Nerves%22&quot;&gt;Nerves&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Ultrasonic+imaging%22&quot;&gt;Ultrasonic imaging&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Vestibular+stimulation%22&quot;&gt;Vestibular stimulation&lt;/searchLink&gt;
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  Data: 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 (&lt;italic&gt;P&lt;/italic&gt; &lt; 0.001). Conversely, the nerve cross‐sectional areas in the upper limb were larger in neuropathy controls than healthy controls (&lt;italic&gt;P&lt;/italic&gt; &lt; 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. [ABSTRACT FROM AUTHOR]
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  Data: &lt;i&gt;Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder&#39;s express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.&lt;/i&gt; (Copyright applies to all Abstracts.)
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        Value: 10.1111/ene.13563
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        Text: English
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    Subjects:
      – SubjectFull: Cerebellar ataxia
        Type: general
      – SubjectFull: Neuropathy
        Type: general
      – SubjectFull: Nerves
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      – SubjectFull: Ultrasonic imaging
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      – SubjectFull: Vestibular stimulation
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              Text: Apr2018
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