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Ultrasonographic evaluation reveals thinning of cervical nerve roots and peripheral nerves in spinal and bulbar muscular atrophy

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Abstract

Background

Ultrasonography (US) is a noninvasive and patient-friendly tool for the evaluation of peripheral nerves. In motor neuron diseases, amyotrophic lateral sclerosis (ALS) has been reported to show the atrophy of peripheral nerves on US. However, the US findings are still unclear in spinal and bulbar muscular atrophy (SBMA), an adult-onset lower motor neuron disease caused by an abnormal CAG repeat expansion in the androgen receptor gene.

Methods

We prospectively recruited and evaluated 11 patients with genetically confirmed SBMA and 9 patients with ALS diagnosed according to the revised El Escorial ALS criteria or the Awaji electrodiagnostic criteria. The C5–C7 cervical nerve roots and the median and ulnar nerves were evaluated ultrasonographically.

Results

The cross-sectional areas (CSAs) of the C6 and C7 nerve roots, the median nerve in the upper arm and forearm, and the ulnar nerve in the upper arm were smaller in patients with SBMA than those in patients with ALS (p < 0.05), whereas the CSAs of the C5 nerve root and the ulnar nerve in the forearm were not smaller.

Conclusions

US showed that the peripheral nerves in patients with SBMA were thinner than those in patients with ALS despite similar degrees of weakness and motor neuron loss. Possible causes include additional sensory nerve involvement and longer disease duration in patients with SBMA than those in patients with ALS.

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Acknowledgements

The authors would like to thank all the patients for their participation in this study.

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Author notes

  1. Tetsuo Komori and Fumiaki Tanaka contributed equally to this work.

Authors and Affiliations

  1. Department of Neurology, National Hospital Organization Hakone Hospital, Odawara, Japan

    Daisuke Watanabe, Tatsuya Abe, Ruriko Kitao, Aya Okuma, Masatoshi Mihara & Tetsuo Komori

  2. Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan

    Daisuke Watanabe, Atsuko Katsumoto, Yukiko Iwahashi, Yuichi Higashiyama, Yosuke Miyaji, Hideto Joki, Hiroshi Doi & Fumiaki Tanaka

  3. Department of Neurology, Tokyo Medical University Ibaraki Medical Center, Ibaraki, Japan

    Hiroshi Tsukamoto

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  1. Daisuke Watanabe
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Corresponding author

Correspondence to Fumiaki Tanaka.

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Ethical approval

The study was approved by the ethics committee of National Hospital Organization Hakone Hospital and Yokohama City University Hospital.

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All patients provided informed consent to participate in the present study prior to enrolment.

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All patients provided informed consent to participate in the present study prior to enrolment.

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The authors declare no competing interests.

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Watanabe, D., Tsukamoto, H., Abe, T. et al. Ultrasonographic evaluation reveals thinning of cervical nerve roots and peripheral nerves in spinal and bulbar muscular atrophy. Neurol Sci 43, 4267–4274 (2022). https://doi.org/10.1007/s10072-022-05969-1

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  • DOI: https://doi.org/10.1007/s10072-022-05969-1

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