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KMID : 1099620160130030170
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Korean Journal of Spine
2016 Volume.13 No. 3 p.170 ~ p.172
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Clinical Experiences of Uncommon Motor Neuron Disease: Hirayama Disease
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Lee Kyoung-Hee
Choi Dae-Seob
Lee Young-Suk
Kang Dong-Ho
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Abstract
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Hirayama disease, juvenile muscular atrophy of the distal upper limb, is a rare disease predominantly affecting the anterior horn cells of the cervical spinal cord in young men. This cervical myelopathy is associated with neck flexion. It should be suspected in young male patients with a chronic history of weakness and atrophy involving the upper extremities followed by clinical stability in few years. Herein, we report 2 cases of Hirayama disease on emphasis of diagnostic approach and describe the pathognomonic findings at flexion magnetic resonance imaging.
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KEYWORD
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Hirayama disease, Juvenile spinal muscular atrophy, Myelopathy
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