title: [Improvement of severe and intravenous immunoglobulin-dependent multifocal motor neuropathy with conduction block after long-term rituximab].
abstract: BACKGROUND
  Some patients suffering from multifocal motor neuropathy with conduction blocks (MMNCB) are still disabled after treatment with intravenous immunoglobulin (IVIg).
  
  
  METHODS
  We report the benefits of a combination of rituximab (RTX) and IVIg in the case of a 72-year-old man with MMNCB.
  
  
  CONCLUSIONS
  Despite an IVIg treatment, the patient had severe motor weakness of the four limbs which limited daily living activity. Azathioprine, mycophenolate mofetyl and cyclophosphamid did not improve the patient's status. Adjunction of rituximab to IVIg therapy increased muscle strength measured on MRC sum score and reduced disability evaluated on ONLS (Overall Neuropathy Limitation Scale) score in the long term (37 months). In spite of the improvement of his neurological status, the patient remained dependent on IVIg.
  
  
  CONCLUSIONS
  RTX could be proposed as a long-term complementary treatment for some severe cases of IVIg-dependent NMMBC. These results must be confirmed in a randomized controlled study.
authors.affiliation: Centre de référence des maladies neuromusculaires et SLA, hôpital de l'Archet, 1, route Saint-Antoine-de-Ginestière, BP 3979, 06202 Nice, cedex 3, France.
authors.forename: A
authors.initials: A
authors.lastname: Michaud
authors.forename: E
authors.initials: E
authors.lastname: Delmont
authors.forename: P-Y
authors.initials: PY
authors.lastname: Jeandel
authors.forename: C
authors.initials: C
authors.lastname: Desnuelle
mesh_terms: D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage*; D003692:Delayed-Action Preparations; D004338:Drug Combinations; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D016472:Motor Neuron Disease / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D009431:Neural Conduction / Q000187:drug effects / Q000502:physiology; D011115:Polyneuropathies / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D000069283:Rituximab; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D003692:Delayed-Action Preparations; D004338:Drug Combinations; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D000069283:Rituximab