PMID- 27105303
OWN - NLM
STAT- MEDLINE
DCOM- 20170630
LR  - 20180209
IS  - 1097-4598 (Electronic)
IS  - 0148-639X (Linking)
VI  - 54
IP  - 6
DP  - 2016 Dec
TI  - Characteristics Of acetylcholine-receptor-antibody-negative myasthenia gravis in 
      a South African cohort.
PG  - 1023-1029
LID - 10.1002/mus.25154 [doi]
AB  - INTRODUCTION: In this study we determined the frequencies of antibodies (Abs) 
      directed against muscle-specific kinase (MuSK) and lipoprotein receptor-related 
      protein 4 (LRP4) in the sera of a South African cohort with acetylcholine 
      receptor (AChR)-antibody-negative generalized MG and determined outcomes to 
      therapies. METHODS: Sera negative by commercial AChR radioimmunoassay (RIA) were 
      tested by MuSK RIA (n = 30; 2006-2012) and AChR, MuSK, and LRP4 RIA with or 
      without cell-based assays (CBA) (n = 53; 2012-2015). RESULTS: AChR-Abs were 
      detected in 4 of 53 and MuSK-Abs in 20 of 83 (24%) cases. Thirty-six of 53 (68%) 
      were triple seronegative (triple-SNMG) for MuSK, AChR, and LRP4-Abs. When 
      compared with triple-SNMG, individuals with MuSK-MG had a younger onset age 
      (P = 0.008), a greater likelihood of African genetic ancestry (P = 0.008), and 
      4-fold higher odds of reaching MGFA grade IVB/V (P = 0.018), but were also 9-fold 
      more likely to reach at least minimal manifestations status after >/=12 months of 
      therapy (P = 0.003). CONCLUSIONS: Individuals with African genetic ancestry and 
      severe bulbar/respiratory AChR-Ab-negative MG are likely to have MuSK-MG, but 
      most respond favorably to maintenance immunotherapies. Muscle Nerve 54: 
      1023-1029, 2016.
CI  - (c) 2016 Wiley Periodicals, Inc.
FAU - Huda, Saif
AU  - Huda S
AD  - Nuffield Department of Clinical Neurosciences, University of Oxford, John 
      Radcliffe Hospital, Oxford, UK.
FAU - Woodhall, Mark R
AU  - Woodhall MR
AD  - Nuffield Department of Clinical Neurosciences, University of Oxford, John 
      Radcliffe Hospital, Oxford, UK.
FAU - Vincent, Angela
AU  - Vincent A
AD  - Nuffield Department of Clinical Neurosciences, University of Oxford, John 
      Radcliffe Hospital, Oxford, UK.
FAU - Heckmann, Jeannine M
AU  - Heckmann JM
AD  - Division of Neurology, E8-74, Department of Medicine, University of Cape Town, 
      South Africa.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20161013
PL  - United States
TA  - Muscle Nerve
JT  - Muscle & nerve
JID - 7803146
RN  - 0 (Autoantibodies)
RN  - 0 (LDL-Receptor Related Proteins)
RN  - 0 (LRP4 protein, human)
RN  - 0 (Receptors, Cholinergic)
RN  - 0 (enhanced green fluorescent protein)
RN  - 147336-22-9 (Green Fluorescent Proteins)
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/*blood
MH  - Cohort Studies
MH  - Female
MH  - Green Fluorescent Proteins/genetics/metabolism
MH  - Humans
MH  - LDL-Receptor Related Proteins/immunology
MH  - Male
MH  - Middle Aged
MH  - Myasthenia Gravis/epidemiology/*immunology
MH  - Odds Ratio
MH  - Receptor Protein-Tyrosine Kinases/*immunology
MH  - Receptors, Cholinergic/*immunology
MH  - South Africa/epidemiology
OTO - NOTNLM
OT  - AChR
OT  - Africa
OT  - LRP4
OT  - MuSK
OT  - cell-based assay
OT  - myasthenia gravis
EDAT- 2016/04/23 06:00
MHDA- 2017/07/01 06:00
CRDT- 2016/04/23 06:00
PHST- 2015/12/29 00:00 [received]
PHST- 2016/04/16 00:00 [revised]
PHST- 2016/04/20 00:00 [accepted]
PHST- 2016/04/23 06:00 [pubmed]
PHST- 2017/07/01 06:00 [medline]
PHST- 2016/04/23 06:00 [entrez]
AID - 10.1002/mus.25154 [doi]
PST - ppublish
SO  - Muscle Nerve. 2016 Dec;54(6):1023-1029. doi: 10.1002/mus.25154. Epub 2016 Oct 13.