HIRAYAMA DISEASE

Course overview

HIRAYAMA IS A RARE NONFAMILIAR MONOMELIC AMYOTROPHY THAT IS ALSO KNOWN AS BENIGN JUVENILE BRACHIAL SPINAL MUSCULAR ATROPHY, JUVENILE ASYMMETRIC SEGMENTAL SPINAL MUSCULAR ATROPHY, JUVENILE MUSCULAR ATROPHY OF THE DISTAL UPPER EXTREMITY, MONOMELIC AMYOTROPHY, AND OBLIQUE AMYOTROPHY. CLASSICALLY, IT PRESENTS WITH MUSCLE ATROPHY AND WEAKNESS OF BILATERAL OR UNILATERAL FOREARMS AND HANDS IN THE ABSENCE OF SENSORY ALTERATIONS, PROGRESSING FOR ONE TO TWO YEARS BEFORE PLATEAUING. THE CONDITION IS CAUSED BY A TIGHT DURAL SAC IN THE CERVICAL CANAL THAT LEADS TO CHRONIC ISCHEMIC CHANGES TO THE ANTERIOR HORN CELLS. ALTHOUGH COMMONLY CONSIDERED A NON-PROGRESSIVE AND SELF-LIMITING DISEASE, THIS HAS BEEN NOTED TO BE A SOURCE OF SIGNIFICANT DISABILITY FOR SOME AFFECTED INDIVIDUALS. MOREOVER, IT IS OFTEN DIAGNOSED AS A MOTOR NEURON DISEASE, WHICH CAN INCREASE THE PSYCHOLOGICAL STRESS FOR AFFECTED PATIENTS. EARLY DIAGNOSIS AND INTERVENTION HAS BEEN SHOWN TO LIMIT THE PROGRESSION AND DECREASE THE DEGREE OF DISABILITY. THIS ACTIVITY REVIEWS THE EVALUATION AND MANAGEMENT OF PATIENTS WITH THIS CONDITION AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN CARING FOR THESE PATIENTS.