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Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis

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Benign monomelic amyotrophy with proximal upper limb involvement: case report.

Marco Antonio Orsini Neves¹, Marcos R G de Freitas, Mariana Pimentel de Mello

¹Clinical Neurology - UNIFESO - Fundação Educacional Serra dos Orgãos (Teresópolis) and Department of Neurology (Neuromuscular Diseases Division) - UFF. orsini@predialnet.com.br

Arquivos De Neuro-Psiquiatria

|August 1, 2007

Summary

Monomelic amyotrophy (MA) is a rare neurological disorder causing muscle wasting in a single limb. This case highlights a 20-year-old man with MA in his right upper limb, stabilizing after four years.

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Area of Science:

Neurology
Clinical Neuroscience

Background:

Monomelic amyotrophy (MA) is a rare neurogenic disorder characterized by muscle wasting confined to one limb.
It typically presents insidiously and progresses over 2-4 years before stabilizing.

Observation:

A case report of a 20-year-old male with slowly progressive amyotrophy and proximal paresis affecting the right upper limb.
Clinical manifestations stabilized four years after onset.

Findings:

Electroneuromyography (EMG) demonstrated denervation and myoffasciculations in multiple muscle groups of the affected right upper limb.
The findings are consistent with neurogenic muscle atrophy localized to a single limb.

Implications:

This case emphasizes the importance of recognizing rare presentations of Monomelic Amyotrophy, particularly in the upper limb.
Further research into the pathophysiology of localized neurogenic amyotrophy is warranted.