title: Laryngeal myasthenia gravis: report of 40 cases.
abstract: Myasthenia gravis, an autoimmune disorder of the neuromuscular junction, is usually recognized because of ocular complaints or generalized weakness. We report a series of 40 patients who presented with dysphonia as their initial and primary complaint. Diagnostic testing included strobovideolaryngoscopy, electromyography (EMG) with repetitive stimulation and Tensilon testing, and laboratory and radiographic evaluation. Strobovideolaryngoscopy most commonly revealed fluctuating impairment of vocal fold mobility, either unilateral or bilateral. EMG detected evidence of neuromuscular junction abnormalities in all patients. Only one patient had evidence of antiacetylcholine receptor (ACh-R) antibodies, but many other abnormalities suggestive of autoimmune dysfunction were present. Pyridostigmine therapy was initiated in 34 patients but was not tolerated in 4. Of the remaining 30 patients, 23 reported improvement of symptoms. We conclude that myasthenia gravis can present with symptoms confined primarily to the larynx and should be included in the differential diagnosis of dysphonia.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
authors.forename: V H
authors.initials: VH
authors.lastname: Mao
authors.forename: M
authors.initials: M
authors.lastname: Abaza
authors.forename: J R
authors.initials: JR
authors.lastname: Spiegel
authors.forename: S
authors.initials: S
authors.lastname: Mandel
authors.forename: M
authors.initials: M
authors.lastname: Hawkshaw
authors.forename: R J
authors.initials: RJ
authors.lastname: Heuer
authors.forename: R T
authors.initials: RT
authors.lastname: Sataloff
mesh_terms: D000328:Adult; D000368:Aged; D002800:Cholinesterase Inhibitors / Q000627:therapeutic use; D004576:Electromyography; D005260:Female; D006801:Humans; D007821:Laryngeal Muscles / Q000503:physiopathology; D007828:Laryngoscopy / Q000379:methods; D007830:Larynx / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000150:complications* / Q000188:drug therapy*; D011729:Pyridostigmine Bromide / Q000627:therapeutic use; D012189:Retrospective Studies; D014832:Voice Disorders / Q000209:etiology* / Q000503:physiopathology*
chemical_list: D002800:Cholinesterase Inhibitors; D011729:Pyridostigmine Bromide