Abstract

Madras Motor Neuron Disease is an anterior horn cell disorder seen in young individuals with prominent cranial nerve involvement and hearing loss. It is sporadic, slowly progressive juvenile onset anterior horn cell disease .In contradiction to classical ALS the clinical course of this disorder is benign and long term survival over decades common. Here we presented a patient with complaints of distal muscle weakness, wasting of upper limbs and sensorineural hearing loss in his medical history. Madras motor neuron disease was diagnosed with neurological examination and EMG findings