PMID- 20544919
OWN - NLM
STAT- MEDLINE
DCOM- 20100817
LR  - 20100726
IS  - 1097-4598 (Electronic)
IS  - 0148-639X (Linking)
VI  - 42
IP  - 2
DP  - 2010 Aug
TI  - Pre- and postsynaptic neuromuscular junction abnormalities in musk myasthenia.
PG  - 283-8
LID - 10.1002/mus.21642 [doi]
AB  - Autoantibodies to muscle-specific kinase (MuSK) can cause myasthenia gravis (MG). 
      The pathophysiological mechanism remains unknown. We report in vitro 
      electrophysiological and histological studies of the neuromuscular junction in a 
      MuSK MG patient. Low levels of presynaptic acetylcholine release and small 
      miniature endplate potentials were found. This combination of pre- and 
      postsynaptic abnormalities was supported by histology, revealing partially 
      denervated postsynaptic areas, and some degeneration of postsynaptic folds. 
      Results suggest that anti-MuSK antibodies reduce the stability of muscle-nerve 
      contact.
FAU - Niks, Erik H
AU  - Niks EH
AD  - Department of Neurology, Leiden University Medical Center, K5Q-114, P.O. Box 
      9600, 2300 RC Leiden, The Netherlands. ehniks@lumc.nl
FAU - Kuks, Jan B M
AU  - Kuks JB
FAU - Wokke, John H J
AU  - Wokke JH
FAU - Veldman, Henk
AU  - Veldman H
FAU - Bakker, Egbert
AU  - Bakker E
FAU - Verschuuren, Jan J G M
AU  - Verschuuren JJ
FAU - Plomp, Jaap J
AU  - Plomp JJ
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Muscle Nerve
JT  - Muscle & nerve
JID - 7803146
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  - Adult
MH  - Autoantibodies/immunology
MH  - Electrophysiology
MH  - Female
MH  - Humans
MH  - Motor Endplate/immunology/physiopathology
MH  - Muscle Weakness/immunology/physiopathology
MH  - Muscle, Skeletal/immunology/physiopathology
MH  - Myasthenia Gravis/immunology/*physiopathology
MH  - Neuromuscular Junction/immunology/*physiopathology
MH  - Receptor Protein-Tyrosine Kinases/*immunology
MH  - Receptors, Cholinergic/*immunology
EDAT- 2010/06/15 06:00
MHDA- 2010/08/18 06:00
CRDT- 2010/06/15 06:00
PHST- 2010/06/15 06:00 [entrez]
PHST- 2010/06/15 06:00 [pubmed]
PHST- 2010/08/18 06:00 [medline]
AID - 10.1002/mus.21642 [doi]
PST - ppublish
SO  - Muscle Nerve. 2010 Aug;42(2):283-8. doi: 10.1002/mus.21642.