Hirayama disease: A case report

Hirayama Disease is a spinal muscular atrophy which presents with varying degrees of unilateral or asymmetric bilateral distal muscle weakness of the upper extremity. It is a rare, non-progressive motor neuron disease, mostly affecting young males. Although considered to have a relatively stationary clinical course after a period of initial progression, it can be functionally impairing in some patients. Here, we present a case of a 20-year-old male who complained of weakness of his right upper extremity for 1 year with difficulty performing activities of daily life. This case highlights the role of radiologist in identifying the subtle findings in cervical spine MRI of patients with such presentation. Moreover, it emphasizes the importance of including flexion imaging MRI sequences in a patient with suspicion of Hirayama disease.

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Publication Details

Journal
Radiology Case Reports
Published
2025-10-15
DOI
https://doi.org/10.1016/j.radcr.2025.09.058
Primary Topic
Cervical and Thoracic Myelopathy
Type
article
Field-Weighted Citation Impact
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article

Hirayama disease: A case report

Bikash Pandey, Ramesh Pokharel, Ashok Raj Pant, Reeha Dhungel
Radiology Case Reports
Cervical and Thoracic Myelopathy
article

Hirayama disease: A case report

Bikash Pandey, Ramesh Pokharel, Ashok Raj Pant, Reeha Dhungel
article en

Abstract

Hirayama Disease is a spinal muscular atrophy which presents with varying degrees of unilateral or asymmetric bilateral distal muscle weakness of the upper extremity. It is a rare, non-progressive motor neuron disease, mostly affecting young males. Although considered to have a relatively stationary clinical course after a period of initial progression, it can be functionally impairing in some patients. Here, we present a case of a 20-year-old male who complained of weakness of his right upper extremity for 1 year with difficulty performing activities of daily life. This case highlights the role of radiologist in identifying the subtle findings in cervical spine MRI of patients with such presentation. Moreover, it emphasizes the importance of including flexion imaging MRI sequences in a patient with suspicion of Hirayama disease.

Radiology Case ReportsVol. 21(1)
Axis Multispecialty Hospital (IN), Mayo Hospital (TH), Mayo Clinic in Florida (US)
Openalex Percentile: Top 54%
Cervical and Thoracic Myelopathy
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Hirayama disease: A case report — Bikash Pandey, Ramesh Pokharel, et al. · Radiology Case Reports (2025) | TGRS Research Map | TGRS