title: [Therapy for anti-MuSK antibody positive myasthenia gravis].
abstract: The authors report the case of a 27-year-old woman with muscle-specific receptor tyrosine kinase antibody positive myasthenia with predominantly ocular and bulbar symptoms. Both edrophonium and low dose (4×30 mg/day) pyridostigmin resulted in cholinergic side effects including fasciculation mainly in the facial and neck muscles, and excessive salivation. The patient responded well to a relatively high dose of chronic corticosteroid treatment (methyprednisolone 64mg/day), but the decrease of the corticosteroid dose below 16 mg/day induced exacerbation of the clinical symptoms. Immunosuppression with azathioprine and methotrexate failed to maintain the clinical improvement. However, plasma exchange was always very effective, and all clinical symptoms improved significantly. The authors conclude that patients with muscle-specific receptor tyrosine kinase antibody positive myasthenia gravis should have an individual treatment protocol differing from those used in patients who do not have this antibody but are positive for acetylcholine-receptor antibody. Identification of the pathogenic antibody in the early stage of myasthenia gravis may help to develop the optimal, individualized treatment strategy, to avoid severe side effects, and to achieve fast improvement.
authors.affiliation: Semmelweis Egyetem, Általános Orvostudományi Kar, Neurológiai Klinika, Molekuláris Neurológiai Központ, Budapest.
authors.forename: Zsuzsanna
authors.initials: Z
authors.lastname: Pál
authors.forename: Judit
authors.initials: J
authors.lastname: Boczán
authors.forename: Benjamin
authors.initials: B
authors.lastname: Bereznai
authors.forename: Gábor
authors.initials: G
authors.lastname: Lovas
authors.forename: Mária Judit
authors.initials: MJ
authors.lastname: Molnár
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D001379:Azathioprine / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008727:Methotrexate / Q000008:administration & dosage; D009157:Myasthenia Gravis / Q000188:drug therapy / Q000276:immunology* / Q000628:therapy*; D010951:Plasma Exchange; D020794:Receptor Protein-Tyrosine Kinases / Q000276:immunology*; D011950:Receptors, Cholinergic / Q000276:immunology*; D017211:Treatment Failure; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; D011950:Receptors, Cholinergic; C095714:MUSK protein, human; D020794:Receptor Protein-Tyrosine Kinases; D001379:Azathioprine; D008727:Methotrexate