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Case Report | Neurology | Volume 15 Issue 6, June 2026 | Pages: 1426 - 1429 | India
Bilateral Hirayama Disease Presenting as Slowly Progressive Distal Upper Limb Amyotrophy: A Rare Case Report
Abstract: Hirayama disease (HD), also known as juvenile muscular atrophy of the distal upper extremity, is a rare benign lower motor neuron disorder caused by dynamic compression of the lower cervical spinal cord during neck flexion. It predominantly affects adolescent and young adult males and typically follows a self-limiting course after 3-5 years. We report a 45-year-old male who presented with a 25-year history of gradually progressive bilateral distal upper limb weakness and marked hand muscle wasting. The patient experienced increasing difficulty in performing fine motor activities, including writing, buttoning clothes, and lifting objects, while proximal muscle strength, lower limb function, sensory examination, cranial nerves, and cerebellar function remained preserved. Electrophysiological studies demonstrated chronic anterior horn cell involvement at the bilateral C7-T1 segments, with fasciculations and chronic neurogenic motor unit changes. Magnetic resonance imaging of the cervical spine demonstrated findings consistent with Hirayama disease. This case is unusual because of its bilateral involvement and prolonged progressive course extending over two decades, unlike the classical self-limiting pattern. It highlights the importance of considering Hirayama disease in the differential diagnosis of distal upper limb amyotrophy and emphasizes the role of electrophysiological studies and dynamic cervical magnetic resonance imaging in establishing an accurate diagnosis.
Keywords: Hirayama disease, Juvenile muscular atrophy of the distal upper extremity, Monomelic amyotrophy, Anterior horn cell disease, Dynamic cervical magnetic resonance imaging (MRI), Distal upper limb amyotrophy, Electromyography (EMG), Cervical flexion myelopathy, Bilateral Hirayama disease, Lower motor neuron syndrome
How to Cite?: Dr. Bollipalli Pavan Teja Chowdhary, Dr. Aman Niranjan Agrawal, Dr. Abhijit Ramdas Rane, "Bilateral Hirayama Disease Presenting as Slowly Progressive Distal Upper Limb Amyotrophy: A Rare Case Report", Volume 15 Issue 6, June 2026, International Journal of Science and Research (IJSR), Pages: 1426-1429, https://www.ijsr.net/getabstract.php?paperid=SR26626130436, DOI: https://dx.doi.org/10.21275/SR26626130436