MRI FINDINGS OF HIRAYAMA DISEASE – A RARE CAUSE OF DISTAL UPPER LIMB WEAKNESS
Dr. Bhavna Arora*, Dr. Sunita Purohit and Dr. Pradeep Goyal
Hirayama disease, also termed non-progressive juvenile spinal muscular atrophy of the distal upper limbs, is a type of cervical myelopathy related to flexion movements of the neck.[1,3] It occurs mainly in young males between the ages of 15 - 25 years. Here we report the MRI findings in two patients- a 16 years old young male presenting with insidious onset weakness, atrophy of left distal upper limb and another patient, a 23 years old male with bilateral distal upper limb weakness since 6 months. Both these young males had atrophy of distal upper limb and Hirayama disease as their clinico-radiological diagnosis. Magnetic resonance imaging (MRI) revealed mild short segmental atrophy and altered intramedullary signal of lower cervical cord in a neutral position. Anterior displacement of the posterior dura compressing the thecal sac and prominent dorsal epidural space with flow voids in flexion MRI study was observed. Both the patients were managed conservatively.
Keywords: Hirayama disease, flexion MRI, cervical cord, dura.
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