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dc.contributor.author | Heckmann, J.M. | |
dc.contributor.author | Hansen, P. | |
dc.contributor.author | Van Toorn, R. | |
dc.contributor.author | Lubbe, Elsabeth (Elsa) | |
dc.contributor.author | Janse van Rensburg, Estrelita | |
dc.contributor.author | Wilmshurst, J. | |
dc.date.accessioned | 2012-07-02T12:05:44Z | |
dc.date.available | 2012-07-02T12:05:44Z | |
dc.date.issued | 2012-06 | |
dc.description.abstract | OBJECTIVES: To report the characteristics of juvenile-onset (<20 years) myasthenia gravis (MG) in Africa. SUBJECTS AND METHOD: 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_US |
dc.description.uri | http://www.samj.org.za | en_US |
dc.identifier.citation | Heckmann, JM, Hansen, P, Van Toorn, R, Lubbe, E, Janse van Rensburg, E & Wilmshurst, J 2012, 'The characteristics of juvenile myasthenia gravis among South Africans', South African Medical Journal, vol. 102, no. 6, pp. 532-536. | en_US |
dc.identifier.issn | 0256-9574 (print) | |
dc.identifier.issn | 2078-5135 (online) | |
dc.identifier.uri | http://hdl.handle.net/2263/19294 | |
dc.language.iso | en | en_US |
dc.publisher | Health and Medical Publishing Group | en_US |
dc.rights | Health and Medical Publishing Group | en_US |
dc.subject | South Africans | en_US |
dc.subject | Juvenile myasthenia gravis | en_US |
dc.title | The characteristics of juvenile myasthenia gravis among South Africans | en_US |
dc.type | Article | en_US |