PMID- 11282149
OWN - NLM
STAT- MEDLINE
DCOM- 20010426
LR  - 20190826
IS  - 0165-5728 (Print)
IS  - 0165-5728 (Linking)
VI  - 115
IP  - 1-2
DP  - 2001 Apr 2
TI  - Multifocal motor neuropathy.
PG  - 4-18
AB  - Multifocal motor neuropathy (MMN) is a recently identified peripheral nerve 
      disorder characterized by progressive, predominantly distal, asymmetric limb 
      weakness mostly affecting upper limbs, minimal or no sensory impairment, and by 
      the presence on nerve conduction studies of multifocal persistent partial 
      conduction blocks on motor but not sensory nerves. The etiopathogenesis of MMN is 
      not known, but there is some evidence, based mostly on the clinical improvement 
      after immunological therapies, that the disease has an immunological basis. 
      Antibodies, mostly IgM, to the gangliosides GM1, and though less frequently, GM2 
      and GD1a, are frequently detected in patients' sera, helping in the diagnosis of 
      this disease. Even if there is some experimental evidence that these antibodies 
      may be pathogenic in vitro, their role in the neuropathy remains to be 
      established. Patients with MMN do not usually respond to steroids or plasma 
      exchange, which may occasionally worsen the symptoms, while the efficacy of 
      cyclophosphamide is limited by its relevant side effects. More than 80% of MMN 
      patients rapidly improve with high dose intravenous immunoglobulin therapy 
      (IVIg). The effect of this therapy is, however, transient and improvement has to 
      be maintained with periodic infusions. A positive response to interferon-beta has 
      been recently reported in a minority of patients, some of whom were resistant to 
      IVIg. Even if many progresses have been made on the diagnosis and therapy of MMN, 
      there are still several issues on the nosological position, etiopathogenesis and 
      long-term treatment of this neuropathy that need to be clarified.
FAU - Nobile-Orazio, E
AU  - Nobile-Orazio E
AD  - "Giorgio Spagnol" Service of Clinical Neuroimmunology, Dino Ferrari Centre, 
      Department of Neurological Sciences, University of Milan, IRCCS Ospedale Maggiore 
      Policlinico, Via Francesco Sforza 35, 20122, Milan, Italy. 
      eduardo.nobile@unimi.it
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - Netherlands
TA  - J Neuroimmunol
JT  - Journal of neuroimmunology
JID - 8109498
RN  - 0 (Autoantibodies)
RN  - 0 (Immunoglobulins, Intravenous)
RN  - 37758-47-7 (G(M1) Ganglioside)
RN  - 77238-31-4 (Interferon-beta)
RN  - 8N3DW7272P (Cyclophosphamide)
SB  - IM
MH  - Autoantibodies/blood
MH  - Chronic Disease
MH  - Cyclophosphamide/therapeutic use
MH  - Diagnosis, Differential
MH  - Electrodiagnosis
MH  - Female
MH  - G(M1) Ganglioside/immunology
MH  - Humans
MH  - Immunoglobulins, Intravenous/therapeutic use
MH  - Interferon-beta/therapeutic use
MH  - Male
MH  - Motor Neuron Disease/*diagnosis/immunology/physiopathology/*therapy
MH  - Neural Conduction
MH  - Peripheral Nervous System Diseases/*diagnosis/immunology/physiopathology/*therapy
MH  - Polyradiculoneuropathy/diagnosis
RF  - 153
EDAT- 2001/04/03 10:00
MHDA- 2001/05/01 10:01
CRDT- 2001/04/03 10:00
PHST- 2001/04/03 10:00 [pubmed]
PHST- 2001/05/01 10:01 [medline]
PHST- 2001/04/03 10:00 [entrez]
AID - S0165572801002661 [pii]
AID - 10.1016/s0165-5728(01)00266-1 [doi]
PST - ppublish
SO  - J Neuroimmunol. 2001 Apr 2;115(1-2):4-18. doi: 10.1016/s0165-5728(01)00266-1.