title: [Successful treatment with rituximab in a refractory Stiff-person syndrome].
abstract: Stiff person syndrome is a rare autoimmune disorder characterized by axial and limb progressive stiffness with surimposed spasms and production of autoantibodies to glutamic acid decarboxylase (GAD). We report a case of a 50-year-old woman who developed a stiff person syndrome resistant to conventional immunosuppressive treatments. Eight months after treatment, indexes of stiffness and spasm frequency improved, while however, the blood and CSF rates of anti-GAD increased. This observation illustrates the complexity of stiff person syndrome immunopathogenesis as well as the relevance of rituximab in this indication.
authors.affiliation: Centre de référence des maladies neuromusculaires et de la SLA, centre hospitalier universitaire de La Timone, 264, rue Saint-Pierre, 13005 Marseille, France. amandine.sevy@ap-hm.fr
authors.forename: A
authors.initials: A
authors.lastname: Sevy
authors.forename: J
authors.initials: J
authors.lastname: Franques
authors.forename: L
authors.initials: L
authors.lastname: Chiche
authors.forename: J
authors.initials: J
authors.lastname: Pouget
authors.forename: S
authors.initials: S
authors.lastname: Attarian
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D018501:Antirheumatic Agents / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007167:Immunotherapy; D008875:Middle Aged; D012074:Remission Induction; D000069283:Rituximab; D016879:Salvage Therapy; D016750:Stiff-Person Syndrome / Q000188:drug therapy*; D017211:Treatment Failure; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018501:Antirheumatic Agents; D000069283:Rituximab