Publication:

Hirayama disease: a rare neurological condition of cervical cord pathology

The disease is characterized by a progressive muscle weakness and wasting of distal upper limb muscles sparing brachioradialis. The pathology is in the lower cervical cord due to forward displacement of dural sac and spinal cord by neck flexion. Repeated neck flexion may result in ischemia of the lower cervical anterior horns. The diagnosis can be confirmed by MRI, EMG. It requires a high degree of suspicion in a young patient presenting with unilateral upper limb distal muscle weakness with no sensory involvement, as early diagnosis will help in preventing the progression of the disease by employing simple measures like usage of a cervical collar. We 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.