PMID- 15261606
OWN - NLM
STAT- MEDLINE
DCOM- 20040830
LR  - 20231213
IS  - 1474-4422 (Print)
IS  - 1474-4422 (Linking)
VI  - 3
IP  - 8
DP  - 2004 Aug
TI  - Immune-mediated components of hereditary demyelinating neuropathies: lessons from 
      animal models and patients.
PG  - 457-65
AB  - Most demyelinating forms of Charcot-Marie-Tooth type 1 (CMT1) neuropathy are 
      slowly progressive and do not respond to anti-inflammatory treatment. In nerve 
      biopsy samples, overt lymphocytic infiltration is absent, but pathological 
      features typical of macrophage-related demyelination have been reported. In mouse 
      models of CMT1, demyelination was substantially reduced when the mutants were 
      backcrossed into an immunodeficient genetic background. A few individual patients 
      with CMT1 respond to anti-inflammatory treatment; however, unlike most patients 
      with CMT1, these patients show accelerated worsening of symptoms, inflammatory 
      infiltrates in nerve biopsies, and clinical features resembling chronic 
      inflammatory demyelinating polyneuropathy as well as CMT1. We conclude that in 
      patients with typical CMT1 and in animal models, a cryptic and mild inflammatory 
      process not responsive to standard anti-inflammatory treatment fosters 
      genetically mediated demyelination.
FAU - Martini, Rudolf
AU  - Martini R
AD  - Department of Neurology, University of Wuerzburg, Germany. 
      rudolf.martini@mail.uni-wuerzburg.de
FAU - Toyka, Klaus V
AU  - Toyka KV
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - England
TA  - Lancet Neurol
JT  - The Lancet. Neurology
JID - 101139309
RN  - 0 (Connexins)
RN  - 0 (Myelin P0 Protein)
RN  - 0 (Myelin Proteins)
RN  - 0 (PMP22 protein, human)
RN  - 0 (Pmp22 protein, mouse)
SB  - IM
MH  - Animals
MH  - Charcot-Marie-Tooth Disease/*genetics/*immunology/physiopathology/therapy
MH  - Clinical Trials as Topic
MH  - Connexins/genetics/immunology/metabolism
MH  - Disease Models, Animal
MH  - Female
MH  - Humans
MH  - Immunotherapy
MH  - Lymphocytes/metabolism/pathology
MH  - Macrophages/metabolism/pathology
MH  - Male
MH  - Myelin P0 Protein/genetics/immunology/metabolism
MH  - Myelin Proteins/*genetics/immunology/metabolism
MH  - Gap Junction beta-1 Protein
RF  - 81
EDAT- 2004/07/21 05:00
MHDA- 2004/08/31 05:00
CRDT- 2004/07/21 05:00
PHST- 2004/07/21 05:00 [pubmed]
PHST- 2004/08/31 05:00 [medline]
PHST- 2004/07/21 05:00 [entrez]
AID - S1474442204008221 [pii]
AID - 10.1016/S1474-4422(04)00822-1 [doi]
PST - ppublish
SO  - Lancet Neurol. 2004 Aug;3(8):457-65. doi: 10.1016/S1474-4422(04)00822-1.