Sri-udomkajorn Somjit, Panichai Patipat, Liumsuwan Sahas
Division of Child Neurology, Queen Sirikit National Institute of Child Health, College of Medicine, Rangsit University, Bangkok, Thailand.
J Med Assoc Thai. 2011 Aug;94 Suppl 3:S152-7.
To study the clinical features, treatment, outcome and factors that affected the outcome of myasthenia gravis (MG) in children.
Children aged 16 years or less with diagnosed myasthenia gravis (MG) seen at Queen Sirikit National Institute of Child Health over a 15-year period with a minimum follow-up of 6 months were reviewed. Demographic, clinical characteristics, treatment and the outcome were analyzed.
One hundred and nineteen MG patients, 100 patients (84%) were ocular MG (OMG) and 19 patients (16%) were generalized MG (GMG). Median age of onset was 4.1 years. OMG patients had the age of onset earlier than GMG patients (p = 0.01). Female to male ratio was 1.8: 1. Ptosis was a clinical feature in 99%, accompanied with ophthalmoplegia in 63%, diplopia in 19.3%, extremity weakness in 13.4%, respiratory muscle weakness in 9%, head tilt in 10.1%, dysphagia in 7.5%, hyperthyroidism in 3.4% and epilepsy in 2.5%. One hundred and six patients who had ptosis as the initial symptom 67% were bilateral ptosis, 33% were unilateral ptosis, 10 patients progressed to GMG in 2 years. Almost all patients were treated with pyridostigmine and prednisolone. At the end of follow-up, 60.5% had pharmacological remission for more than 3 months, 18.5% had complete remission without medication. No definite factors associated with the remission were identified.
OMG is the majority of MG patients and the age of onset is earlier than GMG. Early treatment by prednisolone may have the favorable effect on OMG in the progression to GMG and subsequent involvement to the other eye.
研究儿童重症肌无力（MG）的临床特征、治疗方法、预后以及影响预后的因素。
回顾了在诗丽吉王后国家儿童健康研究所就诊的16岁及以下确诊为重症肌无力（MG）的儿童，这些儿童在15年期间接受了至少6个月的随访。分析了人口统计学、临床特征、治疗方法和预后情况。
119例MG患者中，100例（84%）为眼肌型MG（OMG），19例（16%）为全身型MG（GMG）。发病年龄中位数为4.1岁。OMG患者的发病年龄早于GMG患者（p = 0.01）。男女比例为1.8:1。上睑下垂是99%患者的临床特征，伴有眼肌麻痹的占63%，复视占19.3%，肢体无力占13.4%，呼吸肌无力占9%，头部倾斜占10.1%，吞咽困难占7.5%，甲状腺功能亢进占3.4%，癫痫占2.5%。以眼睑下垂为首发症状的106例患者中，67%为双侧眼睑下垂，33%为单侧眼睑下垂，10例在2年内进展为GMG。几乎所有患者都接受了吡啶斯的明和泼尼松龙治疗。随访结束时，60.5%的患者药物缓解超过3个月，18.5%的患者无需药物完全缓解。未发现与缓解相关的明确因素。
OMG是MG患者的主要类型，发病年龄早于GMG。泼尼松龙早期治疗可能对OMG进展为GMG及随后累及另一只眼有良好效果。
