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Monomelic amyotrophy with proximal upper limb involvement: a case report.
J Med Case Rep. 2016 Mar 17; 10:54.JM

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.

Authors+Show Affiliations

Ministry of Health, Building 1228, Road 4025, Juffair, 340, Kingdom of Bahrain.Department of Neurology, King Fahad Specialist Hospital-Dammam, 6830 Ammar bin Thabit St, Al Muraikabat, Dammam, 32253-3202, Saudi Arabia.Department of Neurology, King Fahad Specialist Hospital-Dammam, 6830 Ammar bin Thabit St, Al Muraikabat, Dammam, 32253-3202, Saudi Arabia.University Medical Center, King Abdullah Medical City, P.O. Box 26671, Adliya, Kingdom of Bahrain. mfalahmd@gmail.com.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

26983673

Citation

Al-Ghawi, Eman, et al. "Monomelic Amyotrophy With Proximal Upper Limb Involvement: a Case Report." Journal of Medical Case Reports, vol. 10, 2016, p. 54.
Al-Ghawi E, Al-Harbi T, Al-Sarawi A, et al. Monomelic amyotrophy with proximal upper limb involvement: a case report. J Med Case Rep. 2016;10:54.
Al-Ghawi, E., Al-Harbi, T., Al-Sarawi, A., & Binfalah, M. (2016). Monomelic amyotrophy with proximal upper limb involvement: a case report. Journal of Medical Case Reports, 10, 54. https://doi.org/10.1186/s13256-016-0843-5
Al-Ghawi E, et al. Monomelic Amyotrophy With Proximal Upper Limb Involvement: a Case Report. J Med Case Rep. 2016 Mar 17;10:54. PubMed PMID: 26983673.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Monomelic amyotrophy with proximal upper limb involvement: a case report. AU - Al-Ghawi,Eman, AU - Al-Harbi,Talal, AU - Al-Sarawi,Adnan, AU - Binfalah,Mohamed, Y1 - 2016/03/17/ PY - 2015/06/13/received PY - 2016/02/19/accepted PY - 2016/3/18/entrez PY - 2016/3/18/pubmed PY - 2016/12/29/medline KW - Amyotrophic lateral sclerosis KW - Electromyography KW - Hirayama disease KW - Magnetic resonance imaging KW - Proximal monomelic amyotrophy SP - 54 EP - 54 JF - Journal of medical case reports JO - J Med Case Rep VL - 10 N2 - 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. SN - 1752-1947 UR - https://www.unboundmedicine.com/medline/citation/26983673/Monomelic_amyotrophy_with_proximal_upper_limb_involvement:_a_case_report_ L2 - https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-016-0843-5 DB - PRIME DP - Unbound Medicine ER -