MG comparison, Ocular vs General

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my first journal reading!^^

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MG comparison, Ocular vs General

  1. 1. Comparison of Clinical Manifestationsbetween Patients withOcular Myasthenia Gravis andGeneralized Myasthenia GravisRoh HS, Lee SY, Yoon JSKorean J Ophthalmol 2011;25(1):1-7 Journal Reading dr. Ersifa Fatimah dr. M. Saiful Islam, SpS(K)
  2. 2. Myasthenia GravisAutoimmune disease characterized bymuscular fatigue due to defectiveneuromuscular transmissionwith the levator palpebralis andextraocular muscles preferentiallyaffected 2
  3. 3. MG patients MG patients> 75% MG pxpresent with 90% will visual generalize complaints within 3 years 50% of px with ocular manifestation develop 80% will generalized generalizeweakness within within 2 years 6 months 3
  4. 4. Immune-related disorder (DM 1, SLE, Sjogren, Graves, Hashimoto, RA, MS)  Blurry vision, tearing, pain, photophobia, reduced visual acuity, decreased tear film, inflammatory soft tissue, exophthalmos, etc Long-term steroid therapy MG:Diplopia / ocular motility defects, ptosis Ocular manifestation 4
  5. 5. Previous studies of MG have not focused onophthalmic manifestations other thanptosis, diplopia, and those caused by the long-term of MGNo known studies comparing the ophthalmicfeatures & complications between patients withocular MG those of generalized MG 5
  6. 6. Materials & Methods Px diagnosed with MG, Jan 1995 - Dec 2007, minimum 1 year follow-up periodDatabase of Dept Neurology & Ophthalmology Yonsei University College of Medicine Data, Diagnosis, & Classification Clinical evaluation, confirmatory Demographic, ophthalmic Osserman score diagnostic test (>/1 from Anti-symptom & sign, associated Gr. I = Ocular gr. Ach Rec Ab, RNST, Neostigmineautoimmune dis., results of test, Pyridostigmine), CT thorax Gr. II-IV = Generalized gr. diagnostic test + contrast Analysis: SPSS ver 12.0; Pearson chi-square, Fisher’s exact test; Wilcoxon rank sum test; significant if p-value < 0.05 6
  7. 7. Results & Discussion• A rare disease, it is often difficult to analyze a large patient population• Median follow up period: 39 months (12-105 months)• Range age at disease onset: 3-71• 65% px develop initial symptoms at age <50 ys.• Mean time interval from the onset of ocular MG to generalization: 3.5 ys 7
  8. 8. Bimodal pattern of age of onset in both genders : early-onset & late-onsetIncidence rates peaking 60 - 80 ys, male predominance in the older age group (Allen et al, 2010) Mean age of disease onset 40.1 ys (male) & 42.8 ys (female) 8
  9. 9. • A significant number of patients reported various ophthalmic symptoms; all of which may be related to the muscular weakness caused by MG.• Symptoms were observed equally in the ocular & generalized MG groups.• 7% of generalize MG group had no ptosis / diplopia 9
  10. 10. Diagnostic TestsThe basic diagnostic methods for MG had lower sensitivities in theocular MG group compared to those in the generalized MG group. 10
  11. 11. The incidence of total autoimmune disease was higher in patients in thegeneralized MG group (p=0.721)Consistent with previous study by Christensen et al, 1995 11
  12. 12. • Anticholinesterase drugs improve symptoms of MG in nearly all ocular and generalized MG patients, but the therapeutic effects are limited. Thus, most patients require additional immunosuppressive treatment such as corticosteroid treatment• Although still controversial, there are several studies which have insisted that systemic steroid treatment in ocular MG patients can prevent disease progression to generalized MG. 12
  13. 13. Long-term corticosteroid effectAn important method for preventing complications in patients receivingcorticosteroids is to limit the total steroid dose. 13
  14. 14. One way to reduce the steroid dose is by performing early thymectomy during the course of treatment.Thymectomy is accepted as an effective treatment for MG, for which itis considered a first line immune treatment.Thymectomy was performed in 65% of generalized MG patients, andthe majority was able to reduce steroid dosage while continuingsymptom improvement. 14
  15. 15. Advice for NeurologistNeurologists may not detect ocular symptoms and signs other than ptosisand diplopia or may not be aware of the ophthalmic complications thatcan result from combined autoimmune diseases and steroid treatment MG patients with ophthalmic signs and symptoms may not receiveimmediate proper ophthalmic management.It is important that patients with ocular MG or generalized MG withophthalmic symptoms should undergo regular eye examinations.Careful attention must be given to generalized MG patients because theyhave a higher risk of ophthalmic and other problems associated withsystemic autoimmune disease and long-term treatment of MG than doocular MG patients 15
  16. 16. Study Limitations• Small sample• Selection bias• Cannot quantitatively compare the efficacies of the treatment to severity of the symptoms due to fluctuations in disease status & treatment response 16
  17. 17. Conclusion• MG patients may experience less common eye problems  can impair quality of life and should not be neglected.• Common autoimmune diseases further supporting ophthalmic complications. In generalized MG, the incidence of autoimmune disease is higher and more variable than that in ocular MG patients; therefore, generalized MG patients should be screened for ophthalmic complications.• Long term steroid treatment in MG patients may also cause complications must be taken into careful consideration 17
  18. 18. Critical Appraisal• Study design: Retrospective• P = Patients with MG• I / C = Ocular MG & Generalized MG• O = difference in clinical manifestation (dx, tx, symptom & sign, related disease)
  19. 19. Best“study designs”for ... 20
  20. 20. Validity• Representative sampleSample size 71, inclusion/exclusion (?)• AllocationRandom (-) Concealed (-)Comparable (+)• MaintenanceAdequate follow up period (+)Equal management (-)Drop out (?) Change group (?)• MeasurementBlind (?) Objective +/-
  21. 21. Importance• Statistical significance  available p-value• Dx: Sensitivity/Specificity (+)• Tx: ?• Related disease, complication: ?• The research was not intended to determine which one is better• Open data  viewer can analyze
  22. 22. Applicability• Patient/ case (+), facility (+)
  23. 23. 24
  24. 24. Thank You 25

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