Monomelic amyotrophy: a rare variant of lower motor neuron disorder (2 clinical cases)

Abstract

Monomelic amyotrophy (MMA) is a rare variant of lower motorneuron disease with benign progression. The disease is characterizedby muscular weakness and atrophy in the hand and forearmon one side or asymmetrical lesion of both upper limbs with thepresence of cold paresis, fasciculations, and fine tremor of thefingers. Sensory disorders, pyramidal symptoms, and involvementof the leg muscles are not typical of it. The final diagnosis ofMMA can be made only on the basis of clinical neurological andelectrophysiological tests as well as long-term follow-up of thepatient. Timely recommendations for correction of the movementpattern and prevention of flexion compression may slowdown the disease progression in some cases and, thereby, improvethe quality of life of patients. The article describes two clinicalcases with a probable diagnosis of MMA.

Keywords

• monomelic atrophy
• hirayama's disease
• motor neuron disease, flexion myelopathy
• amyotrophic lateral sclerosis
• als