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Hirayama's Disease: About a Clinical Observation
Boutayna Touiti1, Zouhayr Souirti1
1Neurology, Hassan II University Hospital, Fez, MAR.
Abstract:
Hirayama disease, also known as non-progressive juvenile spinal muscular atrophy of the upper limbs, brachial monomelic amyotrophy, or benign focal atrophy, affects the C7 D1 myotomes; an electromyogram (EMG) shows neurogenic damage in the C7-C8-T1 territories. It causes weakness and amyotrophy of the distal upper limb. Although it usually occurs on one side only, bilateral symmetric cases of Hirayama disease have occasionally been described. It is a slow, progressive disease, and its evolution can have a good prognosis when detected early in the process. We describe a clinical observation of Hirayama disease, including its clinical and paraclinical peculiarities, and compare it to data from previous studies.
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