Hirayama Disease Presenting to Hand Surgeons - A Case Report -

Journal of the Korean Society for Surgery of the Hand 2008;13(4):317-320.
Published online December 1, 2008.

Suk Ha Jeon, Hyung Ik Shin, Jae Yoon Kim, Moon Sang Chung, Goo Hyun Baek, Young Ho Lee, Hyun Sik Gong

수부 외과의가 감별해야 할 Hirayama 질환 - 1예 보고 -

전숙하, 신형익, 김재윤, 정문상, 백구현, 이영호, 공현식

Abstract

Hirayama disease (Juvenile muscular atrophy of distal upper extremity) is a type of localized motor neuron disease involving lower cervical or upper thoracic segments either unilaterally or bilaterally. The authors report a case of Hirayama disease with a history of minor trauma and unilateral upper extremity involvement, which was initially misdiagnosed as a preganglionic brachial plexus injury. A 17-yr-old boy presented with a one-year history of progressive weakness and atrophy affecting his right hand, especially weak extension of thumb and index finger. The electrodiagnostic findings were compatible with the lesion of the anterior horn cells at the C7, C8, and T1 spinal segments. With neck flexion, cervical magnetic resonance imaging (MRI) showed the anterior shifting of the lower cervical dural sac resulting in the cord compression of those segments. His neurologic symptom did not progress after wearing a cervical collar limiting neck flexion. Hirayama disease is one of differential diagnoses for upper extremity weakness presenting to hand surgeons, and the sagittal MRI in the flexed-neck position is a conformational diagnostic tool.