INTRODUCTION

A 9 yrs old male child born out of non-consang-uinous parentage presented to our department with complaints of hearing impairment, facial weakness, weakness and clawing of both hands with involuntary movements and speech disturbances, weakness was progressive involving the lower limb also but with benign course & with breathlessness. Electromyography shows chronic partial denervation. Mini polymyoclonus, atrophy and fasciculation of tongue, weakness and atrophy of upper extremities with sensorineural deafness were the important clues to the clinical diagnosis. A sub group of motor neuron disease in the younger age group was described from Madras, Tamilnadu State, India by Meenakshi sundaram et al in 1970.