Hirayama disease: a rare neurological condition of cervical
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The present article has been published in the International Journal of Research in Orthopaedics.

Hirayama disease is a rare neurological condition characterized by muscular atrophy of upper extremities unilaterally. It predominantly affects the lower cervical cord. The weakness has an insidious onset and gradual progression for initial couple of years followed by a plateau of muscular atrophy, but no sensory or autonomic involvement.

Herein, the authors report a case of a 25 year old man presenting with progressive distal upper limb muscle wasting, on investigating further diagnosed to have Hirayama disease.

A 25 year old man presented with a 2 year history of slowly progressive weakness of left hand and forearm following a fall from bike. He is not able to lift heavy objects and does not have a good grasp of the left hand. He has neck pain following the fall. No sensory impairment or involvement of any other limb. On examination, left forearm and hand muscles wasting was noted with sparing of brachio-radialis. Thumb abduction and palmar grasp impaired.

Cervical spine X-rays revealeed no gross abnormality. Multiplanar T1, STIR, GRE andT2 sequences through the cervical spine imaged in neutral, flexion and extension of the neck show atrophy and flattening of the spinal cord at C6 and C7 levels (lower cervical cord atrophy) with a minimum AP diameter of about 4.5 mm. The rest of the cord is normal in morphology and signal intensity. But there is no evidence of widening of the posterior epidural space on flexion manoeuvre.

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