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The characteristics of juvenile myasthenia gravis among South Africans

dc.contributor.authorHeckmann, J. M.
dc.contributor.authorHansen, P.
dc.contributor.authorVan Toorn, R.
dc.contributor.authorLubbe, E.
dc.contributor.authorJanse Van Rensburg, E.
dc.contributor.authorWilmshurst, J. M.
dc.date.accessioned2013-07-03T08:19:18Z
dc.date.available2013-07-03T08:19:18Z
dc.date.issued2012-06
dc.identifier.citationHeckmann, J. M. et al. 2012. The characteristics of juvenile myasthenia gravis among South Africans. South African Medical Journal, 102(6):532-536.
dc.identifier.issn2078-5135 (online)
dc.identifier.issn0256-9574 (print)
dc.identifier.urihttp://hdl.handle.net/10019.1/82046
dc.descriptionThe original publication is available at http://www.samj.org.zaen_ZA
dc.description.abstractObjectives. To report the characteristics of juvenile-onset (<20 years) myasthenia gravis (MG) in Africa. Subjects and methods. Six South African centres collected data which included acetylcholine receptor-antibody (AChR-ab) status, delay before diagnosis, MG Foundation of America grade at onset, maximum severity and severity at last visit, therapies, outcomes and complications. Results. We report on 190 individuals with a 4-year median follow-up (interquartile range (IQR) 1 - 8). The median age at symptom onset was 7 years (IQR 4 - 14). Ocular MG (26%) occurred among younger children (mean 5.1 years) compared with those developing generalised MG (74%) (mean 10.2 years) (p=0.0004). Remissions were obtained in 45% of generalised and 50% of ocular MG patients, of whom the majority received immunosuppressive treatment, mainly prednisone. Children with post-pubertal onset had more severe MG, but deaths were infrequent. Thymectomies were performed in 43% of those with generalised MG who suffered greater maximum disease severity (p=0.002); there was a trend towards more remissions in the thymectomy group compared with the non-thymectomy group (p=0.057). There was no racial variation with respect to AChR-ab status, maximum severity, or use of immunosuppression. However, 23% of children of African genetic ancestry developed partial or complete ophthalmoplegia as a complication of generalised MG (p=0.002). Conclusion. Younger children developed ocular MG and older children generalised MG. Persistent ophthalmoplegia developing as a MG complication is not uncommon among juveniles of African genetic ancestry. A successful approach to the management of this complication that causes significant morbidity is, as yet, unclear.en_ZA
dc.format.extentpp. 532-536 : ill.
dc.language.isoen_ZAen_ZA
dc.publisherHealth and Medical Publishing Group (HMPG)
dc.subjectMyasthemia gravis -- South Africa -- Cross-cultural studiesen_ZA
dc.subjectOpthalmoplegia -- South Africa -- Cross-cultural studiesen_ZA
dc.subjectMyasthemia gravis -- South Africa -- Diagnosis -- Researchen_ZA
dc.subjectMyasthemia gravis -- South Africa -- Treatment -- Researchen_ZA
dc.subjectOcular myasthemia gravis in children -- South Africa -- Managementen_ZA
dc.titleThe characteristics of juvenile myasthenia gravis among South Africansen_ZA
dc.typeArticleen_ZA
dc.description.versionPublishers’ version
dc.rights.holderAuthors retain copyrighten_ZA


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