title: [A case of myasthenia gravis with anti-MuSK antibodies showing a dramatic improvement with plasma exchange].
abstract: A 49-year-old woman with seronegative myasthenia gravis (SNMG) was admitted to our hospital with severe respiratory failure, proximal muscle weakness and bulbar palsy. Permanent tracheostomy and continuous mechanical ventilation were performed. At a previous hospital, she was diagnosed as SNMG on the basis of the positive waning during 3 Hz repetitive stimulation of the ulnar nerve, although no acetylcholine receptor antibodies (Ab) were detected by serological examination. Before admission to our hospital, she was treated with corticosteroids, intravenous immunoglobulin and tryptophan column immuno-adsorption therapy without clinical improvement. At our hospital, serological examination detected muscle-specific receptor tyrosine kinase (MuSK) Ab and plasma exchange was performed as treatment. Plasma exchange and subsequent immunomodulating therapy with corticosteroids and tacrolimus showed a dramatic clinical improvement with a marked decline of MuSK Ab level in the serum. These results suggested that plasma exchange should be considered as first choice to treat patients with refractory MuSK Ab-positive MG.
authors.affiliation: Department of Neurology, Kinki University School of Medicine.
authors.forename: Hidekazu
authors.initials: H
authors.lastname: Suzuki
authors.forename: Masakatsu
authors.initials: M
authors.lastname: Motomura
authors.forename: Toshiro
authors.initials: T
authors.lastname: Yoshimura
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Shiraishi
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Mitsui
authors.forename: Takanori
authors.initials: T
authors.lastname: Hasegawa
authors.forename: Masahiko
authors.initials: M
authors.lastname: Atsumi
authors.forename: Masataka
authors.initials: M
authors.lastname: Kitaguchi
authors.forename: Susumu
authors.initials: S
authors.lastname: Kusunoki
mesh_terms: D001323:Autoantibodies / Q000097:blood*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000276:immunology* / Q000628:therapy*; D010951:Plasma Exchange; D020794:Receptor Protein-Tyrosine Kinases / Q000276:immunology*; D011950:Receptors, Cholinergic / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D011950:Receptors, Cholinergic; C095714:MUSK protein, human; D020794:Receptor Protein-Tyrosine Kinases