EAS Publisher | Atypical Presentation of Hirayama Disease with Unusual Proximal Muscle Involvement and Rapid Progression to Distal Muscle: A Case Report

Atypical Presentation of Hirayama Disease with Unusual Proximal Muscle Involvement and Rapid Progression to Distal Muscle: A Case Report

DOI : https://doi.org/10.36349/easjrit.2025.v07i04.002

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Monomelic amyotrophy, known as Hirayama disease, is a rare neurological condition that mostly affects young men. It is characterized by distal muscle atrophy and asymmetric weakness in the upper limbs, which are typically brought on by dynamic cervical spinal cord compression. We report a rare case of Hirayama disease in 2024 who is a 27-year-old male patient presented with weakness of right upper limb for the past 3 months and wrist drop involving right hand for the past three weeks. Clinical examination, lab investigation, nerve conduction study and flexion MRI of cervical spine was done. MRI findings, particularly focal atrophy and asymmetrical spinal cord flattening at the C4-C6 level, were key in confirming the diagnosis and guiding treatment decision. Imaging findings played a pivotal role in the diagnosis and management of Hirayama disease in this patient. His condition improved due to early intervention with immunosuppressive therapy, physiotherapy and regular follow up.

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Lecturer, Dept. of Pharmacology and Therapeutics, Shaheed Monsur Ali Medical College & Hospital, Uttara, Dhaka-1230, Bangladesh

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