Andrews P I, Massey J M, Sanders D B
Department of Pediatrics, Duke University Medical Center, Durham, NC 27710.
Neurology. 1993 May;43(5):977-82. doi: 10.1212/wnl.43.5.977.
We analyzed relationships among pubertal stage at disease onset, sex, disease severity, and acetylcholine receptor antibody (AChR Ab) levels in 46 patients with autoimmune juvenile myasthenia gravis (JMG). Female predominance was least in children with prepubertal disease onset (F:M = 1.3:1) and increased in patients with peripubertal (F:M = 1.8:1) and postpubertal (F:M = 14:1) onset. Seronegative JMG was most common in children with early disease onset: 4 of 9 (44%) with prepubertal, 4 of 22 (18%) with peripubertal, and 0 of 15 (0%) with postpubertal onset were seronegative. The rapid therapeutic response to plasmapheresis was useful in distinguishing some patients with seronegative JMG from those with congenital myasthenia gravis (CMG). The high frequency of seronegative JMG in patients with prepubertal onset indicates that AChR Ab assays do not adequately discriminate between JMG and CMG in young children. Furthermore, the different sex distribution in patients with different pubertal stages at disease onset suggests that sex hormones play an important modulating role in JMG.
我们分析了46例自身免疫性青少年型重症肌无力（JMG）患者发病时的青春期阶段、性别、疾病严重程度和乙酰胆碱受体抗体（AChR Ab）水平之间的关系。青春期前发病的儿童中女性优势最小（女：男 = 1.3：1），青春期发病（女：男 = 1.8：1）和青春期后发病（女：男 = 14：1）的患者中女性优势增加。血清阴性JMG在疾病早期发病的儿童中最为常见：青春期前发病的9例中有4例（44%）、青春期发病的22例中有4例（18%）、青春期后发病的15例中有0例（0%）为血清阴性。血浆置换的快速治疗反应有助于区分一些血清阴性JMG患者与先天性重症肌无力（CMG）患者。青春期前发病患者中血清阴性JMG的高频率表明，AChR Ab检测不能充分区分幼儿中的JMG和CMG。此外，疾病发病时不同青春期阶段患者的不同性别分布表明，性激素在JMG中起重要的调节作用。
