Anlar Banu, Senbil Nesrin, Köse Gülşen, Değerliyurt Aydan
Department of Pediatric Neurology, Hacettepe University, Ankara 06100, Turkey.
Neuromuscul Disord. 2005 May;15(5):355-7. doi: 10.1016/j.nmd.2005.01.010.
Thirty cases of juvenile myasthenia with at least 2 years' follow-up and one measurement of acetylcholine receptor antibody titer were evaluated. Symptoms were initially ocular in 15 (50%) cases; only one developed generalized disease and others remained ocular in 2-24 years' follow-up. Antibodies to acetylcholine receptor (AchRAb) were absent in 12/30 (40%) of all patients initially, but became positive in 5/28 (18%) on further testing in 1-5 years. Spontaneous remission was observed in 6 cases (20%) of whom one became seropositive, and late relapses after 5-11 years' remission were observed in 3 patients with ocular myasthenia, of whom only one developed AchRAbs. Our results show ocular JM has an exceptional tendency to remain confined to these muscles, and delaying the classification of the disease as AchRAb-negative for a few years and repeating serological tests is advisable in the follow-up.
对30例青少年型重症肌无力患者进行了评估,这些患者至少随访了2年且进行了一次乙酰胆碱受体抗体滴度检测。症状最初为眼部症状的有15例(50%);仅1例发展为全身型疾病,其他患者在2至24年的随访中仍为眼部症状。所有患者中最初有12/30(40%)乙酰胆碱受体抗体(AchRAb)阴性,但在1至5年后进一步检测时,有5/28(18%)转为阳性。观察到6例(20%)自发缓解,其中1例血清学转为阳性,3例眼肌型重症肌无力患者在缓解5至11年后出现晚期复发,其中仅1例出现AchRAbs。我们的结果表明,眼肌型青少年型重症肌无力有特别倾向于局限于这些肌肉,在随访中建议将疾病分类为AchRAb阴性推迟数年并重复血清学检测。