Atypical Hirayama disease (case report)
DOI: 10.32364/2587-6821-2024-8-10-7
A.A. Kondratov, A.S. Kotov
M.F. Vladimirskiy Moscow Regional Research and Clinical Institute, Moscow, Russian Federation
Hirayama disease (HD), known as monomelic amyotrophy, is a rare benign disease. Typically, it represents progressive predominantly unilateral atrophy of mainly distal muscles of the upper extremities in young Asian men. Although the disease pathogenesis is still unknown, most scientists assume that a discrepancy between lengths of the spinal cord and the spinal canal caused by a rapid increase in growth, plays a key role in its development. The clinical presentation of HD is characterized by a gradual (3-5 year-long) progression of weakness and muscular atrophy in the distal segments of predominantly one upper limb, symptom deterioration associated with cold exposure and absence of sensory impairments. Sometimes the disease demonstrates atypical symptoms. The main diagnostic method for HD is magnetic resonance imaging of the cervical spine in a flexion position; additionally, stimulation electroneuromyography is performed. Both conservative and surgical therapies are focused on symptom mitigation, rehabilitation and quality of life maintenance.
Keywords: Hirayama disease, myelopathy, monomelic amyotrophy, muscular atrophy.
For citation: Kondratov A.A., Kotov A.S. Atypical Hirayama disease (case report). Russian Medical Inquiry. 2024;8(10):587–591 (in Russ.). DOI: 10.32364/2587-6821-2024-8-10-7
About the authors:
Andrey A. Kondratov — neurologist, M.F. Vladimirskiy Moscow Regional Research and Clinical Institute; 61/2, Shchepkin str., Moscow, 129110, Russian Federation; ORCID iD 0009-0007-4634-4684
Alexey S. Kotov — Dr. Sc. (Med.), Professor, Head of the Department of Neurology, M.F. Vladimirskiy Moscow Regional Research and Clinical Institute; 61/2, Shchepkin str., Moscow, 129110, Russian Federation; ORCID iD 0000-0003-2988-5706
Contact information: Andrey A. Kondratov, e-mail: mrandrewkon@yandex.ru
Financial Disclosure: no authors have a financial or property interest in any material or method mentioned.
There is no conflict of interest.
Received 12.08.2024.
Revised 04.09.2024.
Accepted 27.09.2024.
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