PMID- 31424417
OWN - NLM
STAT- MEDLINE
DCOM- 20200217
LR  - 20200217
IS  - 2214-3602 (Electronic)
IS  - 2214-3599 (Print)
IS  - 2214-3599 (Linking)
VI  - 6
IP  - 3
DP  - 2019
TI  - Ocular Weakness in Myasthenia Gravis: Changes in Affected Muscles are a Distinct 
      Clinical Feature.
PG  - 369-376
LID - 10.3233/JND-190407 [doi]
AB  - INTRODUCTION: In this study we quantitatively describe ocular weakness patterns 
      in myasthenia gravis (MG) to help neurologists in making the clinical diagnosis 
      and to investigate how the current outcome measures reflect ocular weakness in 
      MG. METHODS: We investigated ptosis and diplopia patterns in a retro- and 
      prospective cohort of 306 MG patients. Diplopia was systematically examined by 
      testing extra-ocular muscle (EOM) fatigability in two horizontal and four oblique 
      directions for 60 seconds. RESULTS: Of patients with initial symmetric ptosis, 
      40% developed asymmetric ptosis at the second visit. Changes in form of ptosis 
      occurred less often in seronegative MG patients (50%) than in patients with 
      acetylcholine receptor (AChR) antibodies (70%) or muscle-specific kinase (MuSK) 
      antibodies (69%) (p = 0.038). Of patients with diplopia on the first visit, 
      double vision contained both a vertical and horizontal component in 95%. At the 
      second visit, 83% manifested diplopia in other gaze directions. The mean time (in 
      seconds) to diplopia was 11.6+/-14.0 and the mean time to ptosis was 27.6+/-19.8. 
      Diplopia or ptosis manifested within 30 seconds in 87% and 58%, respectively. 
      Patients who manifested diplopia after 30 seconds, reported no limitations due to 
      diplopia. DISCUSSION: Changes in the gaze directions in which diplopia occurs or 
      ptosis side occur frequently in MG. In diagnostically challenging cases, we 
      recommend testing ptosis and diplopia in multiple gaze directions for 30-60 
      seconds during at least two follow-up visits to maximize the chance of observing 
      changes in ocular weakness patterns.
FAU - de Meel, Robert H P
AU  - de Meel RHP
AD  - Department of Neurology, Leiden University Medical Center, Leiden, The 
      Netherlands.
FAU - Raadsheer, Wouter F
AU  - Raadsheer WF
AD  - Department of Neurology, Leiden University Medical Center, Leiden, The 
      Netherlands.
FAU - van Zwet, Erik W
AU  - van Zwet EW
AD  - Department of Biostatistics, Leiden University Medical Center, Leiden, The 
      Netherlands.
FAU - Tannemaat, Martijn R
AU  - Tannemaat MR
AD  - Department of Neurology, Leiden University Medical Center, Leiden, The 
      Netherlands.
FAU - Verschuuren, Jan J G M
AU  - Verschuuren JJGM
AD  - Department of Neurology, Leiden University Medical Center, Leiden, The 
      Netherlands.
LA  - eng
PT  - Journal Article
PL  - Netherlands
TA  - J Neuromuscul Dis
JT  - Journal of neuromuscular diseases
JID - 101649948
SB  - IM
MH  - Aged
MH  - Blepharoptosis/complications/*diagnosis
MH  - Diplopia/complications/*diagnosis
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Muscle Weakness/complications/*diagnosis
MH  - Myasthenia Gravis/complications/*diagnosis
MH  - Oculomotor Muscles/physiopathology
MH  - Prospective Studies
PMC - PMC6839603
OTO - NOTNLM
OT  - Myasthenia gravis
OT  - diplopia
OT  - fluctuations
OT  - ocular weakness
OT  - ptosis
COIS- R.H.P. de Meel reports no disclosures. W.F. Raadsheer reports no disclosures. 
      E.W. van Zwet reports no disclosures. M.R. Tannemaat reports no disclosures. 
      J.J.G.M. Verschuuren has been involved with MG research sponsored by the Prinses 
      Beatrix Fonds, NIH, FP7 European grant (#602420), consultancies for Argen-X, 
      Alexion and Rapharma. The LUMC received royalties from IBL for antibody tests. 
      All reimbursements were received by the LUMC.
EDAT- 2019/08/20 06:00
MHDA- 2020/02/18 06:00
PMCR- 2019/11/08
CRDT- 2019/08/20 06:00
PHST- 2019/08/20 06:00 [pubmed]
PHST- 2020/02/18 06:00 [medline]
PHST- 2019/08/20 06:00 [entrez]
PHST- 2019/11/08 00:00 [pmc-release]
AID - JND190407 [pii]
AID - 10.3233/JND-190407 [doi]
PST - ppublish
SO  - J Neuromuscul Dis. 2019;6(3):369-376. doi: 10.3233/JND-190407.