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Hirayama Disease: Case Report.
Victor Alves Rodrigues1, Matheus Rocha Pereira Klettenberg1, Luciano Farage2
1Médico Neurologista Pediátrico do Hospital Universitário de Brasília, Brasília, DF, Brasil.
Revista Brasileira De Ortopedia
|August 15, 2022
Summary
Hirayama disease (HD) is a rare motor neuron disorder causing progressive upper limb weakness in young adults. Early diagnosis through clinical suspicion and imaging is crucial for conservative management with a cervical collar.
Area of Science:
- Neurology
- Neuron Science
Background:
- Hirayama disease (HD) is a rare, benign motor neuron disorder typically affecting young men.
- It primarily impacts cervical spinal segments, leading to progressive, often asymmetric, upper limb weakness.
Observation:
- A previously healthy 26-year-old male presented with progressive left upper limb weakness, atrophy, and tremor since age 18.
- Electromyoneurography (EMNG) indicated chronic denervation at C7/C8/T1 nerve roots, worse on the left.
- Cervical spine MRI revealed degenerative changes and spinal cord compression during flexion, with anterior horn signal changes.
Findings:
- Clinical and imaging findings were consistent with Hirayama disease (HD).
- The patient exhibited moderate distal atrophy and weakness, with signs of chronic and active denervation.
- Dynamic MRI demonstrated cervical spinal cord caliber reduction and signal alterations suggestive of HD.
Implications:
- This case highlights the importance of considering Hirayama disease in young patients with focal upper limb atrophy.
- Early and accurate diagnosis, requiring interdisciplinary collaboration, is key for appropriate conservative management, typically involving a soft cervical collar.
- While rare, recognizing HD can prevent misdiagnosis and ensure timely, effective treatment for this self-limiting condition.
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