Hirayama disease - Early MRI diagnosis of subacute medullary ischemia: A case report.
Amyotrophy
Hirayama disease
Magnetic resonance imaging
Journal
Surgical neurology international
ISSN: 2229-5097
Titre abrégé: Surg Neurol Int
Pays: United States
ID NLM: 101535836
Informations de publication
Date de publication:
2020
2020
Historique:
received:
01
04
2020
accepted:
18
04
2020
entrez:
5
6
2020
pubmed:
5
6
2020
medline:
5
6
2020
Statut:
epublish
Résumé
Hirayama disease (HD) is a rare, benign, and self-limiting motor neuron disorder that results in selective motor impairment of the C7-T1 myotomes. It is characterized by progressive, unilateral, or bilateral asymmetric muscle atrophy of the distal upper extremities and myelopathy. A 23-year-old male presented with bilateral atrophy of the thenar/hypothenar eminences/ interosseous muscles, plus left-hand weakness. The cervical MRI documented subacute ischemic damage of the distal cervical cord. To rule out a tumor and reduce questionable cord compression, the patient underwent a C5-C6 anterior cervical discectomy and fusion (ACDF) immediately followed by a laminectomy with durotomy and to obtain a spinal cord biopsy. When the histology confirmed focal cord ischemia consistent with HD, it was clear that both operations were unnecessary. Establishing the diagnosis of HD is based on clinical findings and MRI/flexion MR features which include the demonstration of an increased T2-weighted intramedullary cord signal, enlargement of the posterior epidural space, and segmental spinal cord atrophy. The presence of HD should be recognized as a "nonsurgical entity," and conservative nonsurgical management should be employed.
Sections du résumé
BACKGROUND
BACKGROUND
Hirayama disease (HD) is a rare, benign, and self-limiting motor neuron disorder that results in selective motor impairment of the C7-T1 myotomes. It is characterized by progressive, unilateral, or bilateral asymmetric muscle atrophy of the distal upper extremities and myelopathy.
CASE DESCRIPTION
METHODS
A 23-year-old male presented with bilateral atrophy of the thenar/hypothenar eminences/ interosseous muscles, plus left-hand weakness. The cervical MRI documented subacute ischemic damage of the distal cervical cord. To rule out a tumor and reduce questionable cord compression, the patient underwent a C5-C6 anterior cervical discectomy and fusion (ACDF) immediately followed by a laminectomy with durotomy and to obtain a spinal cord biopsy. When the histology confirmed focal cord ischemia consistent with HD, it was clear that both operations were unnecessary.
CONCLUSION
CONCLUSIONS
Establishing the diagnosis of HD is based on clinical findings and MRI/flexion MR features which include the demonstration of an increased T2-weighted intramedullary cord signal, enlargement of the posterior epidural space, and segmental spinal cord atrophy. The presence of HD should be recognized as a "nonsurgical entity," and conservative nonsurgical management should be employed.
Identifiants
pubmed: 32494390
doi: 10.25259/SNI_151_2020
pii: SNI-11-115
pmc: PMC7265399
doi:
Types de publication
Case Reports
Langues
eng
Pagination
115Informations de copyright
Copyright: © 2020 Surgical Neurology International.
Déclaration de conflit d'intérêts
There are no conflicts of interest.
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