Advanced Hirayama Disease with Successful Improvement of Activities of Daily Living by Operative Reconstruction

  • CHIBA Susumu
    Department of Neurology, School of Medicine, Sapporo Medical University
  • YONEKURA Kazumi
    Department of Neurology, School of Medicine, Sapporo Medical University
  • NONAKA Michio
    Department of Neurology, School of Medicine, Sapporo Medical University
  • IMAI Tomihiro
    Department of Neurology, School of Medicine, Sapporo Medical University
  • MATUMOTO Hiroyuki
    Department of Neurology, School of Medicine, Sapporo Medical University
  • WADA Takuro
    Department of Orthopaedic Surgery, School of Medicine, Sapporo Medical University

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Abstract

Juvenile muscular atrophy of the distal upper extremity (Hirayama disease) is a benign and non-progressive motor neuron disease. Application of a cervical collar is believed to prevent progression of symptoms in the early stages, but there is no effective therapy for the advanced disease. We found that tendon transfer improved the activities of daily living (ADL) of a patient with advanced Hirayama disease. An operative reconstruction can be valuable, even in patients with Hirayama disease who have developed impaired ADL due to extensive intrinsic hand muscle atrophy.

Journal

  • Internal Medicine

    Internal Medicine 43 (1), 79-81, 2004

    The Japanese Society of Internal Medicine

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