title: Monomelic amyotrophy with proximal upper limb involvement: a case report.
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.
  
  
  METHODS
  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.affiliation: Ministry of Health, Building 1228, Road 4025, Juffair, 340, Kingdom of Bahrain.
authors.forename: Eman
authors.initials: E
authors.lastname: Al-Ghawi
authors.affiliation: Department of Neurology, King Fahad Specialist Hospital-Dammam, 6830 Ammar bin Thabit St, Al Muraikabat, Dammam, 32253-3202, Saudi Arabia.
authors.forename: Talal
authors.initials: T
authors.lastname: Al-Harbi
authors.affiliation: Department of Neurology, King Fahad Specialist Hospital-Dammam, 6830 Ammar bin Thabit St, Al Muraikabat, Dammam, 32253-3202, Saudi Arabia.
authors.forename: Adnan
authors.initials: A
authors.lastname: Al-Sarawi
authors.affiliation: University Medical Center, King Abdullah Medical City, P.O. Box 26671, Adliya, Kingdom of Bahrain. mfalahmd@gmail.com.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Binfalah
mesh_terms: D000328:Adult; D002574:Cervical Vertebrae / Q000473:pathology*; D057645:Deltoid Muscle / Q000473:pathology*; D004576:Electromyography; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018482:Muscle, Skeletal / Q000473:pathology*; D026741:Physical Therapy Modalities; D014897:Spinal Muscular Atrophies of Childhood / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D016896:Treatment Outcome
keywords: Amyotrophic lateral sclerosis; Electromyography; Hirayama disease; Magnetic resonance imaging; Proximal monomelic amyotrophy