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Childhood Onset Myasthenia Gravis

NATTE RAKSADAWAN, M.D.*, PONGKIAT KANKIRAWATANA,M.D.*, KAMPANAD BALANKURA, M.D.*, PATCHARIN PRATEEPRATANA, M.D.*, TUMTIP SANGRUCHI, M.D.**, LA-ONGSRI A TCHANEEY ASAKUL, M.D.***

Affiliation : * Division of Neurology, Department of Pediatrics, ** Department of Pathology ***Department of Ophthalmology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand.

Abstract
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.

Keywords : Ocular, Myasthenia Gravis, Childhood, Azathioprine, Thymectomy, Steroid


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MEDICAL ASSOCIATION OF THAILAND
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