The characteristics of juvenile myasthenia gravis among South Africans
The original publication is available at http://www.samj.org.za
Objectives. 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.