Abstract

Motor neuron diseases are a group of progressive neurological disorders that destroy motor neurons that control voluntary muscle activity such as swallowing, walking, speaking and breathing. The common form of motor neuron disease is amyotrophic lateral sclerosis. Neurological examination presents specific signs associated with upper and lower motor neuron degeneration. In the absence of any biological marker, the diagnosis of motor neuron disease is based on clinical features, combined with the results of electromyography. Some patients of motor neuron disease with atypical presentation have been reported in the literature. We presented here a case of motor neuron disease with atypical presentation who had progressive weakness of the neck muscles.