title: Treatment of patients with multifocal motor neuropathy with immunoglobulins in clinical practice: the SIGNS registry.
abstract: OBJECTIVE
  The management of patients with multifocal motor neuropathy (MMN) under everyday clinical conditions has been insufficiently studied. We therefore collected comprehensive observational data on patients with MMN who received intravenous (IV) or subcutaneous (SC) immunoglobulins (IGs) as maintenance therapy.
  
  
  METHODS
  This was a prospective, noninterventional study (registry) in neurological centres (hospitals and offices) throughout Germany.
  
  
  RESULTS
  As of 1 December 2015, 80 patients with MMN were included (mean age 55.4 ± 9.8 years, 67% males, mean disease duration 10.7 ± 10.2 years). The affected limb regions were predominantly distal muscle groups of the upper extremities. On the inflammatory neuropathy cause and treatment (INCAT) scale, 94% of the patients had some disability in the arms and 61% in the legs. At inclusion, 98.8% received IVIG and 1.3% SCIG. Substantial variation was observed between IVIG treatment intervals (every 0.7 to 17.3 weeks) and dosage (0.2-2.1 g/kg body weight received during a single administration; mean monthly dosage, 0.9 g/kg body weight). However, the mean monthly dosage was steady over time. At 1-year follow up, improvement was seen in muscle strength, INCAT and quality of life (QoL) scores (SF-36 questionnaire).
  
  
  CONCLUSIONS
  The management of patients with MMN in everyday clinical practice demonstrates a wide range of absolute dosages and treatment intervals of IG, supporting the recommended practice of determining treatment dose on an individual patient basis. The improvements in muscle strength and reduction in disability, accompanied by increased QoL, strengthen the case for use of IG as a maintenance treatment for MMN.
authors.affiliation: Clinical Neuroimmunology and Neurochemistry, Department of Neurology, Hanover Medical School, Carl-Neuberg-Str. 1, D-30625 Hannover, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Stangel
authors.affiliation: Department for Neurology, St. Josef-Hospital, Ruhr University Bochum, Germany.
authors.forename: Ralf
authors.initials: R
authors.lastname: Gold
authors.affiliation: Institute for Clinical Pharmacology, Medical Faculty, Technical University Dresden, Germany.
authors.forename: David
authors.initials: D
authors.lastname: Pittrow
authors.affiliation: Paediatric Pulmonology, Allergy and Neonatology, Hanover Medical School, Hanover, Germany.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Baumann
authors.affiliation: Paediatric Rheumatology, Immunology and Infectiology, Hospital St. Georg, Leipzig, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Borte
authors.affiliation: Paediatric Rheumatology, Immunology and Infectiology, Hospital St. Georg, Leipzig, Germany.
authors.forename: Maria
authors.initials: M
authors.lastname: Fasshauer
authors.affiliation: Mannheimer Onkologie Praxis, Mannheim, Germany.
authors.forename: Manfred
authors.initials: M
authors.lastname: Hensel
authors.affiliation: Epidemiology, German Rheumatism Research Centre, Berlin, Germany and; Rheumatology and Clinical Immunology, Charité University Hospital, Berlin, Germany.
authors.forename: Dörte
authors.initials: D
authors.lastname: Huscher
authors.affiliation: PIOH - Praxis Internistische Onkologie, Hämatologie, Köln, Germany.
authors.forename: Marcel
authors.initials: M
authors.lastname: Reiser
authors.affiliation: Department of Neurology, University Hospital Würzburg, Germany.
authors.forename: Claudia
authors.initials: C
authors.lastname: Sommer
keywords: autoimmune disease; disability; neuroimmunology; neurology; observational; patient-related outcomes; quality of life; routine care; therapy