Anti-MAG neuropathy is a progressively disabling orphan rare disorder due to a monoclonal immunoglobulin M(IgM) gammopathy displaying reactivity toward MAG, a glycoprotein of the peripheral nervous system. Its prevalence is around 1/100000 and to date, no treatment has proven efficacy in this disease, including rituximab in 2 Randomized Controlled Trails(RCTs).
Disease duration of 5 years or less and documented clinical worsening (clinical or ENMG or disability) over the past 24 months
IgM gammopathy, either MGUS or Waldenstrom Macroglobulinemia (WM)
Demyelinating polyneuropathy according to European Federation of Neurological Societies/Peripheral Nerve Society guidelines for chronic inflammatory demyelinating polyneuropathy on nerve conduction studies.
Anti-MAG titre of 10 000 BTU or more
- Unable to give informed consent
History of severe allergic or anaphylactic reaction to chimeric monoclonal antibody
Hypersensitivity known to one of the compounds of polaramine or methylprednisolone
Previous treatment with rituximab
Centre Hospitalier Universitaire de Saint Etienne
Lead sponsor
Ministry of Health, France
Collaborator