ReviewJournal of neuroimaging : official journal of the American Society of Neuroimaging2022
Hirayama disease: Nosological classification and neuroimaging clues for diagnosis.
Review in Journal of neuroimaging : official journal of the American Society of Neuroimaging, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 20 citations in OpenAlex.
- Silent Damage, Delayed Symptoms: A Case of Breast Cancer Radiation-Induced Lumbosacral Plexopathy.Reports (MDPI) · 2026Article
- Hirayama disease: A case report.Radiology case reports · 2026Article
- Duraplasty with laminoplasty for Hirayama disease: a technical note with a case series.Asian spine journal · 2025Article
- Spinal cord ischemia: The "snake bite sign".World journal of radiology · 2025Article
- Characteristics of Hirayama Disease in Young South Korean Soldiers.Journal of clinical neurology (Seoul, Korea) · 2024Article
- Loss of cervical sagittal alignment worsens the cervical spinal lesions in patients with Hirayama disease.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2023Article
- Surgical management of Hirayama disease in a pediatric patient presenting with severe cervical kyphosis and focal myelopathy: illustrative case.Journal of neurosurgery. Case lessons · 2023Article
- Chiropractic management of a U.S. Veteran with myofascial pain and concurrent distal bimelic amyotrophy (Hirayama disease): a case report.The Journal of the Canadian Chiropractic Association · 2023Article
- Spontaneous conus infarction with "snake-eye appearance" on magnetic resonance imaging: A case report and literature review.World journal of clinical cases · 2023Article
- Hirayama disease: Nosological classification and neuroimaging clues for diagnosis.Journal of neuroimaging : official journal of the American Society of Neuroimaging · 2022Review
- Classical Hirayama Disease Presenting as Progressive Spastic Quadriparesis.Annals of Indian Academy of NeurologyArticle
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Authors and funding
9 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hirayama disease (HD) is a rare, benign, and nonprogressive motor neuron disease (MND) affecting the upper limbs. It usually presents with weakness and amyotrophy in a single upper extremity with an insidious onset between adolescence and the third decade of life. Since its description in 1959, HD has been known under several names and eponyms in Europe and in Asian countries probably due to its heterogeneous clinical features. Thus, the unclear nosological classification makes challenging the differential diagnosis between HD and other neuromuscular conditions, such as MNDs. However, apart from the nosological difficulties and the lack of evidence-based guideline for diagnosis, the neuroimaging is the mainstay for the diagnosis of HD. Indeed, the specific findings on cervical flexion MRI usually lead to a prompt diagnosis. Here, we reviewed the nosological classifications of HD and its neuroimaging features. Also, we report a case of a 18-year-old boy who presented to our Clinic complaining of muscle weakness of the left distal upper limb without other neurological signs. The cervical MRI, in the neutral position, revealed a high T2 signal intensity in the C5-C7 cervical myelomeres as well as the loss of cervical lordosis, whereas, during neck flexion, it showed the anterior displacement of the posterior dura ad the post-gadolinium T1-weighted imaging enhancement of the posterior epidural plexus. These findings are typical for HD allowing the diagnosis as well as the differential diagnosis from other neuromuscular diseases.
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