• Am J Emerg Med · Feb 2024

    Case Reports

    Successful treatment of respiratory failure in Hirayama disease.

    • Nik Sol, Joost Hartman, Mara Ten Kate, Zoé van Kempen, Anneke van der Kooi, Peter Vandertop, Pieter-Roel Tuinman, and van OostenBobBDepartment of Neurology, Amsterdam University Medical Center, the Netherlands..
    • Department of Neurology, Amsterdam University Medical Center, the Netherlands. Electronic address: ni.sol@amsterdamumc.nl.
    • Am J Emerg Med. 2024 Feb 1; 76: 272.e3272.e5272.e3-272.e5.

    AbstractHirayama disease is a self-limiting cervical motor neuron disease, usually affecting the spinal cord at level C7-T1. We share an unusual case of Hirayama disease in a young man affecting roots C4-C6. He presented in coma due to diaphragm weakness and hypercapnic respiratory failure. Diagnosis was achieved via clinical presentation, neurophysiological examination, ultrasonography of the diaphragm and dynamic MR-imaging. Conservative treatment with a cervical collar resulted in remarkable improvement in respiratory and motor function.Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.

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