title: High-dose cyclophosphamide in refractory myasthenia gravis with MuSK antibodies.
abstract: We describe a 48-year-old woman with seronegative myasthenia gravis (MG) and high-titer of anti-MuSK antibody. She had severe bulbar and respiratory weakness with minimal limb weakness for 2 years. Her disease responded poorly to all the conventional immunosuppressive regimens. Treatment with immunoablative dose of cyclophosphamide led to dramatic and sustained remission of her symptoms. High-dose cyclophosphamide is an effective alternative in patients with unusually refractory disease.
authors.affiliation: Department of Neurology and Neurological Sciences, Stanford University, Stanford, California 94305, USA.
authors.forename: Peter T
authors.initials: PT
authors.lastname: Lin
authors.forename: Beth A
authors.initials: BA
authors.lastname: Martin
authors.forename: Ann B
authors.initials: AB
authors.lastname: Weinacker
authors.forename: Yuen T
authors.initials: YT
authors.lastname: So
mesh_terms: D000477:Alkylating Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D000963:Antimetabolites / Q000009:adverse effects / Q000627:therapeutic use; D001379:Azathioprine / Q000009:adverse effects / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use*; D004351:Drug Resistance; D005260:Female; D006801:Humans; D007116:Immunization, Passive; D007166:Immunosuppressive Agents; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000201:enzymology*; D009157:Myasthenia Gravis / Q000188:drug therapy* / Q000276:immunology*; D010951:Plasma Exchange; D011241:Prednisone / Q000627:therapeutic use; D011505:Protein-Tyrosine Kinases / Q000276:immunology / Q000378:metabolism*; D000069283:Rituximab; D016559:Tacrolimus / Q000627:therapeutic use
chemical_list: D000477:Alkylating Agents; D000893:Anti-Inflammatory Agents; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000963:Antimetabolites; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide; D011505:Protein-Tyrosine Kinases; D001379:Azathioprine; D011241:Prednisone; D016559:Tacrolimus