Monomelic amyotrophy with proximal upper limb involvement: A case report

被引：7

作者：

Al-Ghawi E. ^{[1
]}

Al-Harbi T. ^{[2
]}

Al-Sarawi A. ^{[2
]}

Binfalah M. ^{[3
]}

机构：

[1] Ministry of Health, Building 1228, Road 4025, Juffair

[2] Department of Neurology, King Fahad Specialist Hospital-Dammam, 6830 Ammar bin Thabit St, Al Muraikabat, Dammam

[3] University Medical Center, King Abdullah Medical City, P.O. Box 26671, Adliya

来源：

Journal of Medical Case Reports | / 10卷 / 1期

关键词：

Amyotrophic lateral sclerosis; Electromyography; Hirayama disease; Magnetic resonance imaging; Proximal monomelic amyotrophy;

D O I：

10.1186/s13256-016-0843-5

中图分类号：

学科分类号：

摘要：

Background: Monomelic amyotrophy is an uncommon, benign, unilateral disorder of the lower motor neurons, affecting predominantly the hand and forearm muscles. Proximal involvement of the arm and shoulder muscles is an unusual presentation that has been rarely reported in the literature. Case presentation: A 28-year-old white man presented with insidious-onset, slowly progressive, unilateral weakness and atrophy of his left shoulder girdle and deltoid muscles. A neurological examination revealed weakness and atrophy in his left deltoid, infraspinatus and supraspinatus muscles. Electromyography demonstrated an active and chronic neurogenic pattern affecting his left C5 and C6 myotomes; magnetic resonance imaging of his cervical spine was normal. He did well with conservative treatment. Conclusions: Upper limb proximal form of monomelic amyotrophy is a rare clinical entity with a wide differential diagnosis. Physicians, especially neurologists, should be familiar with this benign condition to avoid inappropriately labeling patients as having amyotrophic lateral sclerosis and other disorders with less favorable outcomes. © 2016 Al-Ghawi et al.

引用

共 15 条

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