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Rehabilitation Practice and Science

Translated Title

平山症的臨床診斷與預後探討：病例報告

Abstract

Hirayama disease is a rare disease with unique clinical manifestations. It was first reported by Japanese physician Keizo Hirayama, and tends to develop in young people in their teens and early twenties, predominantly in men. The lower cervical spinal anterior horn cells are usually involved, resulting in asymmetric muscular atrophy or weakness of distal upper extremity. The onset is insidious and gradual. It ends up in a stable status after years of progression. By now the mechanism is still unclear, and two hypotheses are discussed. One is anterior displacement of the cervical dural sac from repeated or sustained neck flexion, resulting in direct lower cervical cord compression. The other is chronic traumatic focal ischemic change in the anterior horn cells caused by repeated neck flexion. It is very important to make the correct diagnosis because Hirayama disease has a benign and self-limited course. It should be distinguished from other motor neuron diseases or cervical myeloradiculopathy, to prevent unnecessary operations or interventions. Electrodiagnostic tests are needed. The cervical dynamic magnetic resonance imaging is also useful to detect cervical cord compression on neck flexion. We reported a 14-year-old boy with Hirayama disease to demonstrate the typical clinical manifestations, electrophysiological tests, and diagnostic imaging studies, and to discuss current therapeutic options. Some experts suggest application of a temporary cervical collar in early stage of disease to prevent hyperflexion of neck and symptom progression. Surgical stabilization has inconsistent outcomes in long term studies, and is only recommended for advanced cases.

Language

Traditional Chinese

First Page

123

Last Page

130

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