Successful treatment of respiratory failure in Hirayama disease

Nik Sol, Joost Hartman, Mara ten Kate,Zoe van Kempen,Anneke van der Kooi, Peter Vandertop, Pieter-Roel Tuinman,Bob van Oosten

AMERICAN JOURNAL OF EMERGENCY MEDICINE(2024)

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摘要
Hirayama 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. (c) 2023 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY license (http:// creativecommons.org/licenses/by/4.0/).
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关键词
Hirayama disease,Respiratory failure,Diaphragm weakness,Ultrasonography,MR imaging
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