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 attention to this rare location of MA, as well as describe some theories concerning its pathophysiology .
Clinical Neurology - UNIFESO - Fundação Educacional Serra dos Orgãos (Teresópolis) and Department of Neurology (Neuromuscular Diseases Division) - UFF. orsini@predialnet.com.br
Marco Antonio Orsini
MA
Neves
Marcos R G de
MR
Freitas
Mariana Pimentel de
MP
Mello
Carlos Henrique
CH
Dumard
Gabriel R de
GR
Freitas
Osvaldo J M
OJ
Nascimento
D000328:Adult; D001132:Arm / Q000503:physiopathology; D004576:Electromyography; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009133:Muscular Atrophy / Q000175:diagnosis* / Q000503:physiopathology; D010291:Paresis / Q000209:etiology*