Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2016 Mar 17:10:54.
doi: 10.1186/s13256-016-0843-5.

Monomelic amyotrophy with proximal upper limb involvement: a case report

Eman Al-Ghawi  1 , Talal Al-Harbi  2 , Adnan Al-Sarawi  2 , Mohamed Binfalah  3
Affiliations
Case Reports

Monomelic amyotrophy with proximal upper limb involvement: a case report

Eman Al-Ghawi et al. J Med Case Rep. .

Abstract

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.

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

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Left shoulder photograph. Atrophy of the deltoid, supraspinatus and infraspinatus muscles
Fig. 2
Fig. 2
Cervical spine magnetic resonance imaging (1.5 Tesla). Sagittal T2 section, demonstrating normal spinal cord and dural canal in the neutral position
See this image and copyright information in PMC

References

    1. Hirayama K, Toyokura Y, Tsubaki T. Juvenile muscular atrophy of unilateral upper extremity: a new clinical entity. Psychiatry Neurol Jpn. 1959;61:2190–7. - PubMed
    1. Peiris JB, Seneviratne KN, Wickremasinghe HR, Gunatilake SB, Gamage R. Non familial juvenile distal spinal muscular atrophy of upper extremity. J Neurol Neurosurg Psychiatry. 1989;52:314–9. doi: 10.1136/jnnp.52.3.314. - DOI - PMC - PubMed
    1. Pradhan S. Bilaterally symmetric form of Hirayama disease. Neurology. 2009;72:2083–9. doi: 10.1212/WNL.0b013e3181aa5364. - DOI - PubMed
    1. De Freitas MR, Nascimento OJ. Benign monomelic amyotrophy: a study of twenty-one cases. Arq Neuropsiquiatr. 2000;58:808–13. doi: 10.1590/S0004-282X2000000500003. - DOI - PubMed
    1. Neves MA, Freitas MR, Mello MP, Dumard CH, Freitas GR, Nascimento OJ. Benign monomelic amyotrophy with proximal upper limb involvement. Arq Neuropsiquiatr. 2007;65:524–7. doi: 10.1590/S0004-282X2007000300032. - DOI - PubMed

Publication types

MeSH terms

Supplementary concepts

LinkOut - more resources