PMID- 16637919
OWN - NLM
STAT- MEDLINE
DCOM- 20060628
LR  - 20151211
IS  - 0065-1427 (Print)
IS  - 0065-1427 (Linking)
VI  - 183
DP  - 2006
TI  - Clinical aspects of neuromuscular transmission disorders.
PG  - 8-11
AB  - Autoimmune disorders of neuromuscular transmission are caused by antibodies (abs) 
      directed against membrane proteins at the motor end-plate. Myasthenia gravis (MG) 
      is due, in most cases, to abs against the nicotinic acetylcholine receptor 
      (AChR). Anti-AChR-positive MG actually includes different disease entities: 
      weakness can be confined to extrinsic ocular muscles or can be generalized; 
      patients with generalized MG (G-MG) can be subdivided on the basis of age of 
      onset, HLA association and thymic pathology. About 15% of G-MG patients are 
      anti-AChR-negative; in a proportion of these cases serum abs against the muscle- 
      specific kinase (MuSK) are found. Anti-MuSK-positive MG is characterized by 
      predominant involvement of bulbar muscles and very low frequency of thymic 
      pathology. The Lambert-Eaton myasthenic syndrome (LEMS) is caused by abs against 
      voltage-gated calcium channels at nerve terminal. LEMS is characterized by muscle 
      weakness and autonomic disturbances and it is paraneoplastic in over 50% of the 
      cases. In neuromyotonia and cramp-fasciculation syndrome, that are thought to be 
      due to anti-voltage-gated potassium channel abs, signs of peripheral nerve 
      hyperexcitability can be associated with CNS features.
FAU - Evoli, Amelia
AU  - Evoli A
AD  - Neuroscience Department, Catholic University, Roma, Italy. a.evoli@rm.unicatt.it
LA  - eng
PT  - Journal Article
PT  - Review
PL  - Denmark
TA  - Acta Neurol Scand Suppl
JT  - Acta neurologica Scandinavica. Supplementum
JID - 0370337
SB  - IM
MH  - Humans
MH  - *Isaacs Syndrome/diagnosis/etiology/therapy
MH  - *Lambert-Eaton Myasthenic Syndrome/diagnosis/etiology/therapy
MH  - *Myasthenia Gravis/diagnosis/etiology/therapy
RF  - 25
EDAT- 2006/04/28 09:00
MHDA- 2006/06/29 09:00
CRDT- 2006/04/28 09:00
PHST- 2006/04/28 09:00 [pubmed]
PHST- 2006/06/29 09:00 [medline]
PHST- 2006/04/28 09:00 [entrez]
AID - ANE606 [pii]
AID - 10.1111/j.1600-0404.2006.00606.x [doi]
PST - ppublish
SO  - Acta Neurol Scand Suppl. 2006;183:8-11. doi: 10.1111/j.1600-0404.2006.00606.x.