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Electric shocks and weakness of the right hand in a young man: Hirayama disease
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Electric shocks and weakness of the right hand in a young man: Hirayama disease
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Journal Article
Electric shocks and weakness of the right hand in a young man: Hirayama disease
2019
Overview
The association of the patient's electric shock-like sensations prompted us to do a dynamic cervical spine MRI study with the neck in positions of flexion and extension: MRI showed that the spinal cord was being compressed—most noticeably at the level of the fifth cervical vertebral body—and that the dorsal epidural space was abnormally expanding while in a flexed position (figure). Hirayama disease—first described by Keizo Hirayama in 1959 as a juvenile, unilateral, muscular atrophy of the upper limb—predominantly affects adolescent men and is thought to be caused by dynamic mechanical or ischaemic injury to the ventral cervical motor neurons. The imaging findings are key to differentiating the disease from more common disorders—such as amyotrophic lateral sclerosis, multiple sclerosis, cervical spondylotic myelopathy, tabes dorsalis, spinal muscular atrophy, or syringomyelia—and allowing for appropriate surgical action, as in our patient.
Publisher
Elsevier Ltd,Elsevier Limited
