Factors affecting outcome in ocular myasthenia gravis

Int J Neurosci. 2018 Jan;128(1):15-24. doi: 10.1080/00207454.2017.1344237. Epub 2017 Jul 17.

Abstract

Aim of the study: 50%-60% of patients with ocular myasthenia gravis (OMG) progress to generalized myasthenia gravis (GMG) within two years. The aim of our study was to explore factors affecting prognosis of OMG and to test the predictive role of several independent clinical variables.

Materials and methods: We reviewed a cohort of 168 Caucasian patients followed from September 2000 to January 2016. Several independent variables were considered as prognostic factors: gender, age of onset, results on electrophysiological tests, presence and level of antibodies against acetylcholine receptors (AChR Abs), treatments, thymic abnormalities. The primary outcome was the progression to GMG and/or the presence of bulbar symptoms. Secondary outcomes were either achievement of sustained minimal manifestation status or worsening in ocular quantitative MG subscore (O-QMGS) or worsening in total QMG score (T-QMGS), assessed by Myasthenia Gravis Foundation of America (MGFA) quantitative scores. Changes in mental and physical subscores of health-related quality of life (HRQoL) were assessed with SF-36 questionnaire. Variance analysis was used to interpret the differences between AChR Ab titers at different times of follow up among the generalized and non-generalized patients.

Results: Conversion to GMG occurred in 18.4% of patients; it was significantly associated with sex, later onset of disease and anti-AChR Ab positivity. Antibody titer above the mean value of 25.8 pmol/mL showed no significant effect on generalization. Sex and late onset of disease significantly affected T-QMGS worsening. None of the other independent variables significantly affected O-QMGS and HRQoL.

Conclusions: Sex, later onset and anti-AChR Ab positivity were significantly associated with clinical worsening.

Keywords: Ocular myasthenia; anti-acetylcholine receptor antibodies; early-onset myasthenia; generalized myasthenia; late-onset myasthenia; outcome measures.

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Aged
  • Aged, 80 and over
  • Autoantibodies / blood*
  • Disease Progression*
  • Female
  • Follow-Up Studies
  • Glucocorticoids / therapeutic use*
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Male
  • Middle Aged
  • Myasthenia Gravis* / blood
  • Myasthenia Gravis* / drug therapy
  • Myasthenia Gravis* / pathology
  • Myasthenia Gravis* / physiopathology
  • Outcome Assessment, Health Care*
  • Receptors, Cholinergic / immunology*
  • Retrospective Studies
  • Severity of Illness Index*
  • Sex Factors
  • Young Adult

Substances

  • Autoantibodies
  • Glucocorticoids
  • Immunosuppressive Agents
  • Receptors, Cholinergic