title: Long-term effects of a multimodal approach including immunoadsorption for the treatment of myasthenic crisis.
abstract: A multimodal treatment protocol with immunoadsorption (IA) as the central element was used in the treatment of myasthenic crisis (MC). Fifteen patients with MC were treated in repeated, uninterrupted 7-day cycles until mobilization with: (i) large-volume IA using an antihuman-IgG adsorber, days 1-5; (ii) intravenous immunoglobulin substitution (0.3-0.5 g/kg body weight [BW]/day), days 5-7; and (iii) immunosuppression with cyclophosphamide (1-2 mg/kg BW/day) and prednisolone (0.5-1 mg/kg BW/day), until remission. Patients required a median of 8 days of mechanical ventilation, 12 days in the intensive care unit, and 35 days of hospitalization. Functional improvement compared to their precrisis condition was attained by 14 of 15 patients. MG severity score improved by a mean of 10 points, quality of life score by 9.8 points, and Karnofsky index by 29 points in 14 of 15 patients. Improvements remained stable and no further crises occurred during long-term follow-up, which averaged 4.4 years. No fatalities due to MC occurred. The results demonstrate that our protocol is a potent therapeutic approach in the treatment of MC.
authors.affiliation: Medical Policlinic, University of Bonn, Bonn, Germany. heike.zeitler@uni-bonn.de
authors.forename: Heike
authors.initials: H
authors.lastname: Zeitler
authors.forename: Gudrun
authors.initials: G
authors.lastname: Ulrich-Merzenich
authors.forename: Lars
authors.initials: L
authors.lastname: Hoffmann
authors.forename: Cornelia
authors.initials: C
authors.lastname: Kornblum
authors.forename: Stephan
authors.initials: S
authors.lastname: Schmidt
authors.forename: Hans
authors.initials: H
authors.lastname: Vetter
authors.forename: Peter
authors.initials: P
authors.lastname: Walger
mesh_terms: D000328:Adult; D000368:Aged; D000888:Antibodies, Anti-Idiotypic / Q000009:adverse effects / Q000627:therapeutic use*; D001323:Autoantibodies / Q000097:blood; D001781:Blood Component Removal / Q000009:adverse effects / Q000379:methods; D003131:Combined Modality Therapy; D005260:Female; D006760:Hospitalization; D006801:Humans; D007074:Immunoglobulin G / Q000009:adverse effects / Q000276:immunology* / Q000627:therapeutic use; D007163:Immunosorbent Techniques; D007164:Immunosorbents / Q000009:adverse effects / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000628:therapy*; D009460:Neurologic Examination; D011978:Receptors, Nicotinic / Q000276:immunology; D012131:Respiratory Insufficiency / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D000888:Antibodies, Anti-Idiotypic; D001323:Autoantibodies; D007074:Immunoglobulin G; D007164:Immunosorbents; D007166:Immunosuppressive Agents; D011978:Receptors, Nicotinic