Hirayama Disease: A 15-Year-Old Male With Progressive Distal Right Upper Extremity Weakness.
Journal
The neurologist
ISSN: 2331-2637
Titre abrégé: Neurologist
Pays: United States
ID NLM: 9503763
Informations de publication
Date de publication:
01 Mar 2023
01 Mar 2023
Historique:
pubmed:
21
5
2022
medline:
10
3
2023
entrez:
20
5
2022
Statut:
epublish
Résumé
Hirayama disease is a rare neuromuscular disorder characterized by abnormal forward displacement of the cervical spinal cord, resulting in focal ischemic changes of anterior horn cells. A 15-year-old male presented with 6 months of progressive right upper extremity weakness. Electromyography/nerve conduction study indicated a chronic neurogenic process involving the C8-T1 myotome. Cervical spine magnetic resonance imaging in the neutral position demonstrated minor disk bulges without significant spinal canal narrowing. With flexion, there was a forward displacement of the dorsal dural sac and marked effacement of the subarachnoid spaces from vertebral levels C5 through C7. In addition, prominent flow voids were now seen in the dorsal epidural space consistent with engorged venous structures. The diagnosis of Hirayama disease requires a high index of suspicion, and imaging should include a series with the neck in a flexed position, as imaging in the neutral position is often unrevealing and the disorder can otherwise easily be missed.
Identifiants
pubmed: 35593905
doi: 10.1097/NRL.0000000000000447
pii: 00127893-202303000-00011
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
126-128Informations de copyright
Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.
Déclaration de conflit d'intérêts
The authors declare no conflict of interest.
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