PMID- 23255823
OWN - NLM
STAT- MEDLINE
DCOM- 20130301
LR  - 20220330
IS  - 1526-632X (Electronic)
IS  - 0028-3878 (Linking)
VI  - 80
IP  - 2
DP  - 2013 Jan 8
TI  - Complete stable remission and autoantibody specificity in myasthenia gravis.
PG  - 188-95
LID - 10.1212/WNL.0b013e31827b907b [doi]
AB  - OBJECTIVES: Patients with myasthenia gravis (MG) are subgrouped as acetylcholine 
      receptor (AChR)-positive, muscle-specific kinase (MuSK)-positive, and 
      AChR/MuSK-negative MG (or double negative [DN]) on the basis of autoantibody 
      assay. We investigated the relationships between autoantibody specificity, main 
      clinical features, and outcome of the disease, in particular the occurrence of 
      complete stable remission (CSR), by means of a retrospective study on a cohort of 
      677 Italian patients with MG. METHODS: A total of 517 (76%) patients with 
      AChR-positive MG, 55 (8%) patients with MuSK-positive MG, and 105 (16%) patients 
      with DN MG were included in the study. Kaplan-Meier and Cox proportional hazard 
      regression analyses were used to evaluate associations between baseline 
      characteristics, antibody specificity, and CSR. RESULTS: Clinical stage at onset 
      and at maximal worsening was more severe for MuSK-positive patients: bulbar 
      impairment at maximal worsening was found in 83.6% of MuSK-positive patients 
      compared with 58.6% of AChR-positive patients and 43.8% of DN patients (p < 
      0.001). Baseline characteristics of AChR-positive and DN patients were similar. 
      CSR was observed in 3.6% of MuSK-positive patients compared with 22.2% of 
      AChR-positive and 21.9% of DN patients. In the whole MG cohort, onset before age 
      40 (hazard ratio [HR] = 1.96, 95% confidence interval [CI] 1.27-3.02, p = 0.002) 
      and ocular and generalized clinical stages at maximal worsening were associated 
      with CSR (ocular, HR = 8.05, 95% CI 1.88-34.53, p = 0.005; generalized, HR = 
      3.71, 95% CI 1.16-11.90, p = 0.023; bulbar, HR = 3.16, 95% CI 1.00-10.05, p = 
      0.051). CONCLUSIONS: MuSK antibodies identify a clinically distinguishable, more 
      severe form of MG since the disease onset, with a lower occurrence of CSR. These 
      features should be considered by the clinician in the management of this 
      particular form of MG.
FAU - Baggi, Fulvio
AU  - Baggi F
AD  - Neurology IV, Neurological Institute Foundation Carlo Besta, Milan, Italy.
FAU - Andreetta, Francesca
AU  - Andreetta F
FAU - Maggi, Lorenzo
AU  - Maggi L
FAU - Confalonieri, Paolo
AU  - Confalonieri P
FAU - Morandi, Lucia
AU  - Morandi L
FAU - Salerno, Franco
AU  - Salerno F
FAU - Bernasconi, Pia
AU  - Bernasconi P
FAU - Montomoli, Cristina
AU  - Montomoli C
FAU - Barberis, Massimo
AU  - Barberis M
FAU - Mantegazza, Renato
AU  - Mantegazza R
FAU - Antozzi, Carlo
AU  - Antozzi C
LA  - eng
PT  - Journal Article
DEP - 20121219
PL  - United States
TA  - Neurology
JT  - Neurology
JID - 0401060
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  - Age of Onset
MH  - Antibody Specificity
MH  - Autoantibodies/*analysis
MH  - Cohort Studies
MH  - Data Interpretation, Statistical
MH  - Disease Progression
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Myasthenia Gravis/*immunology/pathology/*therapy
MH  - Neurosurgical Procedures
MH  - Plasma Exchange
MH  - Receptor Protein-Tyrosine Kinases/immunology
MH  - Receptors, Cholinergic/immunology
MH  - Retrospective Studies
MH  - Sex Characteristics
MH  - Thymus Gland/pathology/surgery
MH  - Treatment Outcome
EDAT- 2012/12/21 06:00
MHDA- 2013/03/02 06:00
CRDT- 2012/12/21 06:00
PHST- 2012/12/21 06:00 [entrez]
PHST- 2012/12/21 06:00 [pubmed]
PHST- 2013/03/02 06:00 [medline]
AID - WNL.0b013e31827b907b [pii]
AID - 10.1212/WNL.0b013e31827b907b [doi]
PST - ppublish
SO  - Neurology. 2013 Jan 8;80(2):188-95. doi: 10.1212/WNL.0b013e31827b907b. Epub 2012 
      Dec 19.