[Neurophysiological parameters in myasthena gravis in children in diagnostic and therapeutic view].

Przeglad lekarski Pub Date : 2016-01-01
Sławomir Kroczka, Katarzyna Stasiak, Marek Kaciński
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Abstract

Background: Myasthenia gravis (MG) is an autoimmunologic disorder. It is characterized by various clinical symptoms and their dependency upon the exertion and the rest as well.

Material and methods: Between 2002-20014 in the Neurophysiology Laboratory at the Chair of Pediatric and Adolescent Neurology, Jagiellonian University in Krakow, the electrophysiological repetitive nerve stimulation study were performed in 44 children. The clinical picture and positive electrophysiological test were the ground to diagnose MG in 19 of them (12 girls and 7 boys). The mean age at onset of MG in the examined group was 12.8 years. In 5 patients the diagnosis was completed using edrophonium test. In 14patients the titer of AchRAb was also tested. In 18 patients with MG the radiological examinations of the chest were performed. In 14/19 patients with MG the control electrophysiological testing was performed 2 to 8 months after the first one.

Results: The gen-ralized MG was diagnosed in 14 patients, and ocular in 2 of them. The amplitude of electro-physiological testing was normal during the first response in all patients, but the decrement of amplitude 4:1 in patients with MG was 26% to 88%. In 3 patients with MG the persisted thymus, while in 7 hypertrophy of thymus, in 2 thymoma nad inl thyinflammation of the thymus was detected. In 5 children the result of chest examination was normal. Among 5/8 patients (62.5%) with positive AChRAb, in one ocular MG was diagnosed, in the other 7 generalized MG. The titer of AChRAb was between 0.4 and 30.8 nmol/l (mean 9.44 nmol/l), and the decrement of amplitude 4:1 was 22% to 58%.. In the treatment pirydostygmine bromide (Mestinon) was used in all children, however in 4 of them together with azathioprine, and in 7 with steroids. In the treatment of myasthenic crisis in 5 patients plasmapheresis was performed. In 42% of patients thymectomy was performed during the first year after diagnosis. The clinical remission was succeed in 90,9% patients. The electrophysiological control examination detected the decrement of amplitude 4:1 in 1% to 80% (mean 36%). The electrophysiological remission, correlating with clinical remission was achieved in 2 patients. In 6/10 patients with clinical remission, the decrement of amplitude 4:1 in electrophysiological examination continued to be at the level similar to initial examination. In 1 patient remission was complete allowing significant reduction of doses of Mestinon, even though in the electrophysiological test the decrement was 80%.

Conclusions: (1) Among hospitalized children, the generalized myasthenia was the most common. (2) Neurophysiological studies plays still fundamental role in diagnostic methods in the diagnosis of myasthenia gravis in children. (3) The correlation of the clinical state and electrophysiological results was not established.

[儿童重症肌无力的神经生理参数诊断与治疗观点]。
背景:重症肌无力是一种自身免疫性疾病。它的特点是各种临床症状及其对运动和休息的依赖性。材料和方法:2002-20014年间,在克拉科夫雅盖隆大学儿科和青少年神经病学主席神经生理学实验室,对44名儿童进行了电生理重复神经刺激研究。其中19例(女孩12例,男孩7例)以临床表现和阳性电生理检查为诊断依据。实验组MG发病的平均年龄为12.8岁。5例患者通过碘离子试验完成诊断。在14例患者中也检测了AchRAb滴度。18例MG患者行胸部影像学检查。14/19 MG患者在第一次电生理检查后2 ~ 8个月进行对照电生理检查。结果:14例患者诊断为广泛性MG,其中2例为眼部MG。所有患者首次反应时的电生理测试波幅均正常,但MG患者的波幅以4:1递减26% ~ 88%。3例胸腺持续,7例胸腺肥大,2例胸腺瘤,1例胸腺炎症。5例患儿胸部检查结果正常。AChRAb阳性的5/8例(62.5%)中,1例诊断为眼部MG, 7例诊断为广泛性MG。AChRAb滴度在0.4 ~ 30.8 nmol/l之间(平均9.44 nmol/l),幅度4:1下降22% ~ 58%。在治疗中,所有儿童均使用吡哆苯甲胺(Mestinon),但其中4例与硫唑嘌呤联合使用,7例与类固醇联合使用。对5例重症肌无力危象患者行血浆置换术。42%的患者在诊断后的第一年内进行了胸腺切除术。90%的患者临床缓解成功。电生理对照检查在1% ~ 80%(平均36%)范围内检测到幅度4:1的衰减。2例患者达到电生理缓解,与临床缓解相关。在6/10临床缓解的患者中,电生理检查的幅度减小幅度为4:1,继续保持在与初检相似的水平。1例患者完全缓解,允许显著减少Mestinon剂量,即使在电生理测试中减少了80%。结论:(1)住院患儿以全身性重症肌无力最为常见。(2)在儿童重症肌无力的诊断方法中,神经生理学研究仍具有基础性作用。(3)临床状态与电生理结果的相关性未建立。
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