PMID- 16155434
OWN - NLM
STAT- MEDLINE
DCOM- 20060314
LR  - 20191026
IS  - 1350-7540 (Print)
IS  - 1350-7540 (Linking)
VI  - 18
IP  - 5
DP  - 2005 Oct
TI  - Neuromuscular junction autoimmune disease: muscle specific kinase antibodies and 
      treatments for myasthenia gravis.
PG  - 519-25
AB  - PURPOSE OF REVIEW: Some of the 20% of myasthenia gravis patients who do not have 
      antibodies to acetylcholine receptors (AChRs) have antibodies to muscle specific 
      kinase (MuSK), but a full understanding of their frequency, the associated 
      clinical phenotype and the mechanisms of action of the antibodies has not yet 
      been achieved. Moreover, some patients do not respond well to conventional 
      corticosteroid therapy. Here we review recent clinical and experimental studies 
      on MuSK antibody associated myasthenia gravis, and summarize the results of newer 
      treatments for myasthenia gravis. RECENT FINDINGS: MuSK antibodies are found in a 
      variable proportion of AChR antibody negative myasthenia gravis patients who are 
      often, but not exclusively, young adult females, with bulbar, neck, or 
      respiratory muscle weakness. The thymus histology is normal or only very mildly 
      abnormal. Surprisingly, limb or intercostal muscle biopsies exhibit no reduction 
      in AChR numbers or complement deposition. However, patients without AChR or MuSK 
      antibodies appear to be similar to those with AChR antibodies and may have 
      low-affinity AChR antibodies. A variety of treatments, often intended to enable 
      corticosteroid doses to be reduced, have been used in all types of myasthenia 
      gravis with some success, but they have not been subjected to randomized clinical 
      trials. SUMMARY: MuSK antibodies define a form of myasthenia gravis that can be 
      difficult to diagnose, can be life threatening and may require additional 
      treatments. An improved AChR antibody assay may be helpful in patients without 
      AChR or MuSK antibodies. Clinical trials of drugs in other neuroimmunological 
      diseases may help to guide the treatment of myasthenia gravis.
FAU - Vincent, Angela
AU  - Vincent A
AD  - Neurosciences Group, Weatherall Institute of Molecular Medicine and Department of 
      Clinical Neurology, University of Oxford, Oxford, UK. Angela.vincent@imm.ox.ac.uk
FAU - Leite, Maria Isabel
AU  - Leite MI
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - England
TA  - Curr Opin Neurol
JT  - Current opinion in neurology
JID - 9319162
RN  - 0 (Autoantibodies)
RN  - 0 (Receptors, Cholinergic)
RN  - EC 2.7.10.1 (MUSK protein, human)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
SB  - IM
MH  - Autoantibodies
MH  - Humans
MH  - Myasthenia Gravis/*immunology/*therapy
MH  - Neuromuscular Junction Diseases/immunology
MH  - Receptor Protein-Tyrosine Kinases/*immunology
MH  - Receptors, Cholinergic/*immunology
MH  - Thymus Gland/pathology
RF  - 43
EDAT- 2005/09/13 09:00
MHDA- 2006/03/15 09:00
CRDT- 2005/09/13 09:00
PHST- 2005/09/13 09:00 [pubmed]
PHST- 2006/03/15 09:00 [medline]
PHST- 2005/09/13 09:00 [entrez]
AID - 00019052-200510000-00007 [pii]
AID - 10.1097/01.wco.0000180660.57801.3f [doi]
PST - ppublish
SO  - Curr Opin Neurol. 2005 Oct;18(5):519-25. doi: 10.1097/01.wco.0000180660.57801.3f.