PMID- 37531447
OWN - NLM
STAT- MEDLINE
DCOM- 20231216
LR  - 20231228
IS  - 1468-1331 (Electronic)
IS  - 1351-5101 (Linking)
VI  - 31
IP  - 1
DP  - 2024 Jan
TI  - Clinical characteristics and treatment outcomes in patients with 
      double-seronegative myasthenia gravis.
PG  - e16022
LID - 10.1111/ene.16022 [doi]
AB  - BACKGROUND AND PURPOSE: Double-seronegative myasthenia gravis (dSNMG) is defined 
      as myasthenia gravis (MG) without detectable or low affinity antibodies to 
      acetylcholine receptor (AChR) and muscle-specific kinase (MuSK). There are 
      limited data on detailed clinical features and outcomes after treatment in dSNMG 
      patients. The aim was to describe the clinical characteristics and outcomes in 
      dSNMG patients based on MG scales. METHODS: A retrospective study was performed 
      of patients diagnosed with MG who had negative AChR or MuSK antibodies and they 
      were compared with an AChR-positive MG cohort. Correlations were made with data 
      from the first and last clinic visits, between demographics, clinical 
      characteristics, treatment and disease severity, based on the Myasthenia Gravis 
      Foundation of America category, Myasthenia Gravis Impairment Index (MGII), 
      Patient Acceptable Symptom State and simple single question (SSQ). RESULTS: 
      Eighty patients met the inclusion criteria for dSNMG. The baseline MGII and SSQ 
      scores in the dSNMG cohort showed no significant differences from the AChR group 
      (p = 0.94 and p = 0.46). The dSNMG cohort MGII and SSQ scores improved 
      significantly at the last clinical evaluation (p = 0.001 and p = 0.047). The MGII 
      improvement in the AChR cohort was significantly better (p = 0.003). CONCLUSIONS: 
      The initial severity of dSNMG based on clinical scores is similar to 
      antibody-positive MG patients. There is significant clinical improvement in dSNMG 
      patients after therapy, measured in the last clinical evaluation. This supports 
      an immune pathophysiology of many dSNMG patients.
CI  - (c) 2023 The Authors. European Journal of Neurology published by John Wiley & Sons 
      Ltd on behalf of European Academy of Neurology.
FAU - Martinez-Harms, Rodrigo
AU  - Martinez-Harms R
AUID- ORCID: 0000-0003-1679-395X
AD  - Ellen and Martin Prosserman Centre for Neuromuscular Diseases, Toronto General 
      Hospital, Toronto, Ontario, Canada.
AD  - University Health Network, University of Toronto, Toronto, Ontario, Canada.
FAU - Barnett, Carolina
AU  - Barnett C
AD  - Ellen and Martin Prosserman Centre for Neuromuscular Diseases, Toronto General 
      Hospital, Toronto, Ontario, Canada.
AD  - University Health Network, University of Toronto, Toronto, Ontario, Canada.
FAU - Alcantara, Monica
AU  - Alcantara M
AD  - Ellen and Martin Prosserman Centre for Neuromuscular Diseases, Toronto General 
      Hospital, Toronto, Ontario, Canada.
AD  - University Health Network, University of Toronto, Toronto, Ontario, Canada.
FAU - Bril, Vera
AU  - Bril V
AUID- ORCID: 0000-0002-5805-4883
AD  - Ellen and Martin Prosserman Centre for Neuromuscular Diseases, Toronto General 
      Hospital, Toronto, Ontario, Canada.
AD  - University Health Network, University of Toronto, Toronto, Ontario, Canada.
LA  - eng
PT  - Journal Article
DEP - 20230816
PL  - England
TA  - Eur J Neurol
JT  - European journal of neurology
JID - 9506311
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
RN  - 0 (Autoantibodies)
RN  - 0 (Receptors, Cholinergic)
SB  - IM
MH  - Humans
MH  - Retrospective Studies
MH  - *Receptor Protein-Tyrosine Kinases
MH  - Autoantibodies
MH  - *Myasthenia Gravis/drug therapy/diagnosis
MH  - Receptors, Cholinergic
MH  - Treatment Outcome
OTO - NOTNLM
OT  - double-seronegative myasthenia gravis
OT  - myasthenia gravis
OT  - outcome
OT  - treatment
EDAT- 2023/08/02 19:15
MHDA- 2023/12/17 09:41
CRDT- 2023/08/02 14:13
PHST- 2023/07/07 00:00 [revised]
PHST- 2023/04/17 00:00 [received]
PHST- 2023/07/30 00:00 [accepted]
PHST- 2023/12/17 09:41 [medline]
PHST- 2023/08/02 19:15 [pubmed]
PHST- 2023/08/02 14:13 [entrez]
AID - 10.1111/ene.16022 [doi]
PST - ppublish
SO  - Eur J Neurol. 2024 Jan;31(1):e16022. doi: 10.1111/ene.16022. Epub 2023 Aug 16.