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

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.
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.
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.
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
  • 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)

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research network!

Choose Username:
Verify Password: