Successful treatment of respiratory failure in Hirayama disease

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 ac...

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Bibliographic Details
Published in:The American journal of emergency medicine 2024-02, Vol.76, p.272.e3-272.e5
Main Authors: Sol, Nik, Hartman, Joost, Ten Kate, Mara, van Kempen, Zoé, van der Kooi, Anneke, Vandertop, Peter, Tuinman, Pieter-Roel, van Oosten, Bob
Format: Article
Language:English
Subjects:
Anesthesia
Atrophy
Catheters
Compression therapy
Consciousness
Continuous positive airway pressure
Diaphragm
Diplopia
Electromyography
Emergency medical care
Epidural
Magnetic resonance imaging
Mitochondrial DNA
Motor neuron diseases
Mutation
Neck
Ostomy
Patients
Spinal cord
Tracheotomy
Ultrasonic imaging
Ventilators
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Summary: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.
ISSN:0735-6757
1532-8171
1532-8171
DOI:10.1016/j.ajem.2023.11.049