PMID- 22892950
OWN - NLM
STAT- MEDLINE
DCOM- 20130129
LR  - 20120910
IS  - 1473-6551 (Electronic)
IS  - 1350-7540 (Linking)
VI  - 25
IP  - 5
DP  - 2012 Oct
TI  - Myasthenia and the neuromuscular junction.
PG  - 523-9
LID - 10.1097/WCO.0b013e3283572588 [doi]
AB  - PURPOSE OF REVIEW: Myasthenic syndromes are distinct disorders at the 
      neuromuscular junction, most often with well characterized autoimmune or genetic 
      pathology. New aspects of the dysfunctions give insight into the normal 
      neuromuscular function in addition to giving therapeutic clues and tailoring the 
      therapy to the pathophysiology in individual patients. RECENT FINDINGS: Patients 
      with myasthenia gravis and congenital myasthenic syndromes should be further 
      classified into distinct subgroups. Myasthenia gravis with low-affinity 
      acetylcholine receptor (AChR) antibodies and myasthenia gravis with antibodies to 
      the postsynaptic low-density lipoprotein receptor-related protein 4 represent new 
      groups, whereas a myasthenia gravis subgroup without any detectable antibodies 
      still persists. Myasthenia gravis with antibodies against muscle-specific kinase 
      (MuSK) is, due to new reports, now as established as AChR-myasthenia gravis 
      regarding disease mechanisms and recommended therapy. SUMMARY: Myasthenic 
      syndromes and myasthenia gravis are well characterized disorders. The prognosis 
      is generally good, apart from paraneoplastic Lambert-Eaton myasthenic syndrome. 
      However, patients need long-term symptomatic and immunoactive treatment, this 
      treatment to be balanced against present and potential side effects. New and more 
      selective treatment is needed, especially for severe generalized disease. Well 
      controlled long-term studies of sufficient power are much wanted, but new therapy 
      has often to be tried in patients before high-class evidence of effect on 
      myasthenia gravis has been published.
FAU - Gilhus, Nils E
AU  - Gilhus NE
AD  - Department of Clinical Medicine, University of Bergen, Bergen, Norway. 
      nils.gilhus@helse-bergen.no
LA  - eng
PT  - Journal Article
PT  - Review
PL  - England
TA  - Curr Opin Neurol
JT  - Current opinion in neurology
JID - 9319162
RN  - 0 (Autoantibodies)
RN  - 0 (Immunosuppressive Agents)
SB  - IM
MH  - Animals
MH  - Autoantibodies/immunology
MH  - Humans
MH  - Immunosuppressive Agents/therapeutic use
MH  - Immunotherapy
MH  - Myasthenia Gravis/immunology/*physiopathology/therapy
MH  - Neuromuscular Junction/immunology/*physiopathology
MH  - Plasma Exchange
MH  - Thymectomy
MH  - Thymus Gland/physiology
EDAT- 2012/08/16 06:00
MHDA- 2013/01/30 06:00
CRDT- 2012/08/16 06:00
PHST- 2012/08/16 06:00 [entrez]
PHST- 2012/08/16 06:00 [pubmed]
PHST- 2013/01/30 06:00 [medline]
AID - 10.1097/WCO.0b013e3283572588 [doi]
PST - ppublish
SO  - Curr Opin Neurol. 2012 Oct;25(5):523-9. doi: 10.1097/WCO.0b013e3283572588.