Abstract | PURPOSE OF REVIEW: 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.
|
Authors | Angela Vincent, Maria Isabel Leite |
Journal | Current opinion in neurology
(Curr Opin Neurol)
Vol. 18
Issue 5
Pg. 519-25
(Oct 2005)
ISSN: 1350-7540 [Print] England |
PMID | 16155434
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
|
Chemical References |
- Autoantibodies
- Receptors, Cholinergic
- MUSK protein, human
- Receptor Protein-Tyrosine Kinases
|
Topics |
- Autoantibodies
- Humans
- Myasthenia Gravis
(immunology, therapy)
- Neuromuscular Junction Diseases
(immunology)
- Receptor Protein-Tyrosine Kinases
(immunology)
- Receptors, Cholinergic
(immunology)
- Thymus Gland
(pathology)
|