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Rehabilitation Practice and Science

Translated Title

青少年遠端上肢肌萎縮：病例報告

Abstract

Juvenile muscular amyotrophy of distal upper limbs (Hirayama disease) is a rare disease predominantly affecting the anterior horn cells of the cervical cord in young men. It is a kind of cervical myelopathy characterized by insidious onset of unilateral distal dominant upper limbs muscle weakness and atrophy due to anterior cervical cord compression. It is difficult to differentiate this disease from other diseases with similar symptoms such as motor neuron disease. Cervical magnetic resonance (MR) study in flexed position is helpful to confirm the diagnosis.We reported a 21-year-old man who complained of slowly progressed muscle atrophy and weakness of right hand and forearm after right shoulder stretching injury three years ago. Electrodiagnostic study revealed acute and chronic denervative change in the right side atrophied muscles. Routine cervical MR images showed high signal intensity over the anterior horn cells of the lower cervical cord. With the suspicion of Hirayama disease, flexion MR was performed and the striking and pathognomonic picture of anterior shifting of posterior dura at the lower cervical spinal canal was noted. He received neck collar therapy and vitamin B12 supplement. No further progression of symptoms was noted at the 3 months follow-up study. Though Hirayama disease is spontaneous arrested, early diagnosis is necessary because early cervical collar application by preventing neck flexion has been shown to stop disease progression. Surgical managements are preserved to late stage. In cases of early onset of distal upper limb weakness with cold paresis and contractile fasciculation, the finding of asymmetric lower cervical cord atrophy on routine cervical MR study raise the suspicion of Hirayama disease. A flexion MR study should be performed to confirm the diagnosis.

Language

English

First Page

107

Last Page

114

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