Benign monomelic amyotrophy with proximal upper limb involvement: case report

Monomelic amyotrophy (MA) is a rare condition in which neurogenic amyotrophy is restricted to an upper or lower limb. Usually sporadic, it usually has an insidious onset with a mean evolution of 2 to 4 years following first clinical manifestations, which is, in turned, followed by stabilization. We report a case of 20-years-old man who presented slowly progressive amyotrophy associated with proximal paresis of the right upper limb, which was followed by clinical stabilization 4 years later. Eletroneuromyography revealed denervation along with myofasciculations in various muscle groups of the right upper limb. We call atention to this rare location of MA, as well as describe some theories concerning its pathophysiology .

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Bibliographic Details
Main Authors: Neves,Marco Antonio Orsini, Freitas,Marcos R.G. de, Mello,Mariana Pimentel de, Dumard,Carlos Henrique, Freitas,Gabriel R. de, Nascimento,Osvaldo J.M.
Format: Digital revista
Language:English
Published: Academia Brasileira de Neurologia - ABNEURO 2007
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2007000300032
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Description
Summary:Monomelic amyotrophy (MA) is a rare condition in which neurogenic amyotrophy is restricted to an upper or lower limb. Usually sporadic, it usually has an insidious onset with a mean evolution of 2 to 4 years following first clinical manifestations, which is, in turned, followed by stabilization. We report a case of 20-years-old man who presented slowly progressive amyotrophy associated with proximal paresis of the right upper limb, which was followed by clinical stabilization 4 years later. Eletroneuromyography revealed denervation along with myofasciculations in various muscle groups of the right upper limb. We call atention to this rare location of MA, as well as describe some theories concerning its pathophysiology .