PMID- 15042294
OWN - NLM
STAT- MEDLINE
DCOM- 20040811
LR  - 20181113
IS  - 0028-2804 (Print)
IS  - 0028-2804 (Linking)
VI  - 75
IP  - 4
DP  - 2004 Apr
TI  - ["Seronegative" myasthenia with antibodies against muscle-specific 
      tyrosine-kinase].
PG  - 362-7
AB  - About 10-20% of patients with generalized myasthenia gravis do not have 
      acetylcholin receptor (AChR) antibodies and are referred to as having 
      "seronegative" myasthenia. Recently, antibodies to muscle-specific kinase (MuSK), 
      which mediates aggregation of AChR, were detected in half of these patients. We 
      investigated six patients with seronegative myasthenia and identified two 
      characteristic cases with MuSK antibodies. A 56-year-old woman developed ptosis 
      and double vision followed by bulbar symptoms and respiratory insufficiency. She 
      showed moderate response to treatment with pyridostigmine and no response to 
      prednisolone, azathioprine, or intravenous immunoglobulin but good response to 
      several plasma exchanges. A 46-year-old man with exercise-induced weakness of the 
      upper limbs and mild bulbar and respiratory symptoms had a milder course for 9 
      years. These cases highlight the clinical spectrum of MuSK-positive myasthenia 
      and the differences to AChR-positive myasthenia with predominant bulbar symptoms 
      and often severe symptoms with respiratory insufficiency. Finally, they 
      illustrate the therapeutic difficulties with less response to pyridostigmine and 
      immunosuppressive drugs.
FAU - Hain, B
AU  - Hain B
AD  - Klinik und Poliklinik fur Neurologie, Martin-Luther-Universitat Halle-Wittenberg, 
      Halle/Saale, Deutschland. Berit.Hain@medizin.uni-halle.de
FAU - Hanisch, F
AU  - Hanisch F
FAU - Deschauer, M
AU  - Deschauer M
LA  - ger
PT  - Case Reports
PT  - English Abstract
PT  - Journal Article
TT  - "Seronegative" Myasthenie mit Antikorpern gegen die muskelspezifische 
      Tyrosin-Kinase.
PL  - Germany
TA  - Nervenarzt
JT  - Der Nervenarzt
JID - 0400773
RN  - 0 (Antibodies)
RN  - 0 (Biomarkers)
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  - Antibodies/*blood/*immunology
MH  - Biomarkers/blood
MH  - Diagnosis, Differential
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Myasthenia Gravis/blood/diagnosis/*enzymology/*immunology
MH  - Receptor Protein-Tyrosine Kinases/*blood/*immunology
MH  - Receptors, Cholinergic/*blood/*immunology
EDAT- 2004/03/26 05:00
MHDA- 2004/08/12 05:00
CRDT- 2004/03/26 05:00
PHST- 2004/03/26 05:00 [pubmed]
PHST- 2004/08/12 05:00 [medline]
PHST- 2004/03/26 05:00 [entrez]
AID - 10.1007/s00115-003-1672-5 [doi]
PST - ppublish
SO  - Nervenarzt. 2004 Apr;75(4):362-7. doi: 10.1007/s00115-003-1672-5.