Childhood onset myasthenia gravis.
The authors share experiences in taking care of 27 cases of childhood onset myasthenia gravis (MGS). In all cases, the diagnosis was confirmed by a combination of clinical examination and Neostigmine test. The majority (92%) had localized ocular myasthenia with median onset of symptoms at 33 months of age. About 24 per cent of them progressed to generalized MGS. A few (8%) presented with respiratory failure that required ventilatory support with onset of symptoms at about 22 months. Thymectomy was performed in 10 cases. Complete and partial remissions were achieved in about 70 per cent and 26 per cent of cases respectively with the combination of an immunosuppressant (azathioprine) and a Cholinesterase inhibitor (pyridostigmine). None experienced a myasthenic crisis with proper management and good follow-up using the above combinations.
Raksadawan
Natte
N
Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Kankirawatana
Pongkiat
P
Balankura
Kampanad
K
Prateepratana
Patcharin
P
Sangruchi
Tumtip
T
Atchaneeyasakul
La-Ongsri
LO
D000293:Adolescent; D017677:Age Distribution; D017668:Age of Onset; D001379:Azathioprine / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D009157:Myasthenia Gravis / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology*; D011379:Prognosis; D012307:Risk Factors; D012720:Severity of Illness Index; D017678:Sex Distribution; D013256:Steroids / Q000008:administration & dosage; D013785:Thailand / Q000453:epidemiology; D013934:Thymectomy / Q000379:methods; D016896:Treatment Outcome
D013256:Steroids; D001379:Azathioprine