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IP International Journal of Orthopaedic Rheumatology


Hirayama Disease: A rare case report with involvement of cervical spine


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Abstract

Introduction: Hirayama disease is a type of cervical myelopathy, also termed non progressive juvenile spinal muscular atrophy mainly involves forearm and hand means distal part of upper extremities. It’s mainly affects the young male.it is benign motor neuron disease because mainly become stationary within 5 years of onset. Diagnosis is based on clinical findings and dynamic flexion MRI images showing anterior displacement of the posterior wall of dura mater and venous congestion in the epidural space. The patient of hirayama disease is mainly treated conservatively. Surgery is required only in severe cases.
Patient: A 19 year man came with a history of insidious onset of weakness in both the hands, left side followed by right of 4 years duration. He noticed weakness in the left hand muscles which was gradually progressed to the fore arm muscles. Within 6 months he noticed similar complaints in the right hand also, which was progressed gradually to the fore arm muscles. He had also noticed atrophy of muscles of hand and fore arm which was gradually progressive in nature. We present a cases meeting both clinical criteria and dynamic radiological criteria which suggests diagnosis of Hirayama disease. Patient has stabilized spontaneously over the course, and MRI scans show that typical changes have disappeared. On follow up no progression of disease occurred and condition remained stable.
Conclusions: Our case suggest that the condition of the patient with Hirayama stabilizes naturally; the natural course of the disease seems to recommend conservative treatment, for conservative treatment, a cervical collar is used which avoids neck flexion and prevents worsening of disease and provides remission. Surgical treatment is limited to patients with severe progressive disease.

Keyword:
Hirayama disease, Cervical spine, Monomelic amyotrophy

Doi No:-10.18231