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[Ocular involvement in MuSK antibody-positive myasthenia gravis].

AbstractBACKGROUND:
Antibodies (Ab) against the acetylcholine receptor (AChR) are found in 80 - 90 % of generalized myasthenia gravis (MG), but only in 50 % of pure ocular MG. Furthermore, Ab against the muscle-specific receptor tyrosine kinase (MuSK) were detected in 38 - 54 % of patients with AChR-Ab-negative ("seronegative") MG, but not in pure ocular MG.
METHODS:
2 case reports have been analysed: two patients (42 years old and 61 years old) with primary ocular MG were studied. Both patients complained of fluctuating bilateral ptosis and double vision lasting for 2 to 3 months.
RESULTS:
AChR-Ab were negative but MuSK-Ab were clearly positive. In spite of treatment with pyridostigmine and prednisolone, the symptoms in patient 1 generalized within 4 weeks causing dysphagia, limb muscle weakness and respiratory crisis. Symptoms immediately responded to plasmapheresis. Patient 2 clearly improved under treatment with pyridostigmine, methylprednisone, and azathioprine. There was no generalization within a course of 12 months.
CONCLUSIONS:
Both cases clearly indicate that MuSK-Ab can be found in primary isolated ocular MG lasting for 2 to 3 months. In a primary ocular manifestation of MG the detection of MuSK might indicate the risk for subsequent generalization and might have implications for immunosuppressive therapy. Therefore, seronegative MG requires testing for MuSK-Ab.
AuthorsV Bau, F Hanisch, B Hain, S Zierz
JournalKlinische Monatsblätter für Augenheilkunde (Klin Monbl Augenheilkd) Vol. 223 Issue 1 Pg. 81-3 (Jan 2006) ISSN: 0023-2165 [Print] Germany
Vernacular TitleOkuläre Beteiligung bei MuSK-Antikörper-positiver Myasthenia gravis.
PMID16418940 (Publication Type: Case Reports, English Abstract, Journal Article)
Chemical References
  • Autoantibodies
  • Receptors, Cholinergic
  • Prednisolone
  • MUSK protein, human
  • Receptor Protein-Tyrosine Kinases
  • Pyridostigmine Bromide
  • Azathioprine
  • Methylprednisolone
Topics
  • Adult
  • Autoantibodies (analysis)
  • Autoimmune Diseases (diagnosis, drug therapy, immunology)
  • Azathioprine (adverse effects, therapeutic use)
  • Drug Therapy, Combination
  • Female
  • Humans
  • Methylprednisolone (adverse effects, therapeutic use)
  • Middle Aged
  • Myasthenia Gravis (diagnosis, drug therapy, immunology)
  • Prednisolone (adverse effects, therapeutic use)
  • Pyridostigmine Bromide (adverse effects, therapeutic use)
  • Receptor Protein-Tyrosine Kinases (immunology)
  • Receptors, Cholinergic (immunology)

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