Biomaterial Database

Multifocal motor neuropathy. 2013

Raquel Guimarães-Costa, and Francesco Bombelli, and Jean-Marc Léger

National Referral Center for Rare Neuromuscular Diseases, Institut Hospitalo-Universitaire de Neurosciences, University Hospital Pitié-Salpêtrière, University Pierre et Marie Curie, Paris VI, Paris, France.

OBJECTIVE Multifocal motor neuropathy (MMN) remains a difficult issue for neurologists, as its clinical and electrophysiological presentation may be atypical, and because no alternative treatment to periodic immunoglobulin infusions has been assessed in its long-term management. This review intends to summarize the most recent advances in the diagnosis and treatment of MMN. RESULTS Recent reports have focused on atypical onset and unusual clinical presentation. Several sophisticated electrophysiological techniques, as triple stimulation, may help establish the presence of conduction blocks, as well as MRI findings. A recent immunological study focused on the detection of serum IgM binding to NS6S heparin disaccharide. In another research article, it was proposed that the use of combinatorial glycoarray or ELISA may increase the diagnostic sensitivity of antiglycolipid antibody testing. Subcutaneous immunoglobulin may represent an interesting alternative option to intravenous immunoglobulin. Lastly, recently reported open-label clinical trials with complement inhibitors and anti-CD20 monoclonal antibody may constitute a first step for further developments. CONCLUSIONS Diagnostic criteria for MMN are well established, but challenging situations still occur. Progresses in neurophysiologic and other laboratory tests may help in clarifying doubtful diagnoses. Current research into the pathophysiology of MMN is required to determine the future treatment targets.

UI	MeSH Term	Description	Entries
D009431	Neural Conduction	The propagation of the NERVE IMPULSE along the nerve away from the site of an excitation stimulus.	Nerve Conduction,Conduction, Nerve,Conduction, Neural,Conductions, Nerve,Conductions, Neural,Nerve Conductions,Neural Conductions
D011115	Polyneuropathies	Diseases of multiple peripheral nerves simultaneously. Polyneuropathies usually are characterized by symmetrical, bilateral distal motor and sensory impairment with a graded increase in severity distally. The pathological processes affecting peripheral nerves include degeneration of the axon, myelin or both. The various forms of polyneuropathy are categorized by the type of nerve affected (e.g., sensory, motor, or autonomic), by the distribution of nerve injury (e.g., distal vs. proximal), by nerve component primarily affected (e.g., demyelinating vs. axonal), by etiology, or by pattern of inheritance.	Polyneuropathy, Acquired,Polyneuropathy, Critical Illness,Polyneuropathy, Familial,Polyneuropathy, Inherited,Polyneuropathy, Motor,Acquired Polyneuropathies,Acquired Polyneuropathy,Critical Illness Polyneuropathies,Critical Illness Polyneuropathy,Familial Polyneuropathies,Familial Polyneuropathy,Inherited Polyneuropathies,Inherited Polyneuropathy,Motor Polyneuropathies,Motor Polyneuropathy,Polyneuropathies, Acquired,Polyneuropathies, Critical Illness,Polyneuropathies, Familial,Polyneuropathies, Inherited,Polyneuropathies, Motor,Polyneuropathy
D004187	Disaccharides	Oligosaccharides containing two monosaccharide units linked by a glycosidic bond.	Disaccharide
D006493	Heparin	A highly acidic mucopolysaccharide formed of equal parts of sulfated D-glucosamine and D-glucuronic acid with sulfaminic bridges. The molecular weight ranges from six to twenty thousand. Heparin occurs in and is obtained from liver, lung, mast cells, etc., of vertebrates. Its function is unknown, but it is used to prevent blood clotting in vivo and vitro, in the form of many different salts.	Heparinic Acid,alpha-Heparin,Heparin Sodium,Liquaemin,Sodium Heparin,Unfractionated Heparin,Heparin, Sodium,Heparin, Unfractionated,alpha Heparin
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001323	Autoantibodies	Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.	Autoantibody
D016756	Immunoglobulins, Intravenous	Immunoglobulin preparations used in intravenous infusion, containing primarily IMMUNOGLOBULIN G. They are used to treat a variety of diseases associated with decreased or abnormal immunoglobulin levels including pediatric AIDS; primary HYPERGAMMAGLOBULINEMIA; SCID; CYTOMEGALOVIRUS infections in transplant recipients, LYMPHOCYTIC LEUKEMIA, CHRONIC; Kawasaki syndrome, infection in neonates, and IDIOPATHIC THROMBOCYTOPENIC PURPURA.	Antibodies, Intravenous,Human Intravenous Immunoglobulin,IV Immunoglobulin,IVIG,Intravenous Antibodies,Intravenous Immunoglobulin,Intravenous Immunoglobulins,Alphaglobin,Endobulin,Flebogamma DIF,Gamimmune,Gamimmune N,Gamimune,Gamimune N,Gammagard,Gammonativ,Gamunex,Globulin-N,IV Immunoglobulins,Immune Globulin Intravenous (Human),Immune Globulin, Intravenous,Immunoglobulins, Intravenous, Human,Intraglobin,Intraglobin F,Intravenous IG,Intravenous Immunoglobulins, Human,Iveegam,Modified Immune Globulin (Anti-Echovirus Antibody),Privigen,Sandoglobulin,Venimmune,Venoglobulin,Venoglobulin-I,Globulin N,Human Intravenous Immunoglobulins,Immunoglobulin, Human Intravenous,Immunoglobulin, IV,Immunoglobulin, Intravenous,Immunoglobulins, Human Intravenous,Immunoglobulins, IV,Intravenous Immune Globulin,Intravenous Immunoglobulin, Human,Venoglobulin I
D016896	Treatment Outcome	Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.	Rehabilitation Outcome,Treatment Effectiveness,Clinical Effectiveness,Clinical Efficacy,Patient-Relevant Outcome,Treatment Efficacy,Effectiveness, Clinical,Effectiveness, Treatment,Efficacy, Clinical,Efficacy, Treatment,Outcome, Patient-Relevant,Outcome, Rehabilitation,Outcome, Treatment,Outcomes, Patient-Relevant,Patient Relevant Outcome,Patient-Relevant Outcomes