Ocular presentation of myasthenia gravis: A natural history cohort

Muscle Nerve. 2018 Apr;57(4):622-627. doi: 10.1002/mus.25971. Epub 2017 Oct 6.

Abstract

Introduction: There are limited data on the natural history of untreated myasthenia gravis (MG) with ocular presentation.

Methods: We analyzed 93 patients from symptom onset who presented to the Birmingham Midlands Eye Centre (BMEC) between January 2004 and July 2015. We used multiple stepwise logistic regression to identify predictive factors of generalization and Kaplan-Meier analysis on time to generalization.

Results: Forty-six percent of patients developed generalized symptoms during the study period. Median time to generalization was 7 months. Time to generalization was earlier in patients seropositive for acetylcholine receptor (AChR) antibody (median 5 months vs. 21 months, P < 0.0001) and bilateral ptosis at onset (P = 0.015). Multivariate analysis identified AChR seropositivity [hazard ratio (HR) 5.03; 95% confidence interval (CI) 1.48-17.14; P = 0.001] and disease onset < 50 years (HR 3.58; 95% CI 1.18-10.90; P = 0.035) as risk factors for generalization.

Discussion: As patients were steroid-naive before generalization, our cohort approximated the natural history of the condition. Muscle Nerve 57: 622-627, 2018.

Keywords: generalization; immunosuppression; natural history; ocular myasthenia; outcomes; steroids.

MeSH terms

  • Autoantibodies / immunology
  • Disease Progression
  • Electromyography
  • Female
  • Humans
  • Kaplan-Meier Estimate
  • Male
  • Middle Aged
  • Myasthenia Gravis / immunology
  • Myasthenia Gravis / physiopathology*
  • Oculomotor Muscles / physiopathology*
  • Orbital Diseases / immunology
  • Orbital Diseases / physiopathology*
  • Proportional Hazards Models
  • Receptors, Cholinergic / immunology

Substances

  • Autoantibodies
  • Receptors, Cholinergic