Güneş Ayfer Sakarya, Genç Hülya Maraş, Yalçın Emek Uyur, Yılmaz Vuslat, Direskeneli Güher Saruhan, Kara Bülent
Division of Child Neurology, Department of Pediatrics, Kocaeli University Faculty of Medicine, Kocaeli, Turkey.
Department of Physiology, İstanbul University Faculty of Medicine, İstanbul, Turkey.
Turk J Pediatr. 2019;61(5):794-797. doi: 10.24953/turkjped.2019.05.023.
Sakarya Güneş A, Maraş Genç H, Uyur Yalçın E, Yılmaz V, Saruhan Direskeneli G, Kara B. Acute ophthalmoparesis and persistent mydriasis: expanding the clinical spectrum of anti-GQ1b positive cranial neuropathy in a 5.5-year-old girl. Turk J Pediatr 2019; 61: 794-797. Acute ophthalmoparesis without ataxia (AO) is an atypical form of Miller- Fisher syndrome (MFS) and is rare in children. Anti-GQ1b antibodies can be detected in patients with AO, as in MFS. A 5.5-year-old girl had total ophthalmoparesis, blurred vision, ptosis, diplopia and mydriasis non-reactive to light or near stimuli with preserved consciousness and deep tendon reflexes. She had no ataxia. Cerebrospinal fluid (CSF) examination and cranial MRI were normal. Serum antiGQ1b antibodies were positive. She was diagnosed with AO and intravenous Immunoglobulin (IVIG) was ordered, 400 mg/ kg/day, for 5 days. Ophthalmoparesis and blurred vision improved in a few weeks. At the end of the first year, mydriasis still persisted, but improved and became responsive to near stimuli. Pupillary involvement may be seen in approximately 50% of MFS patients, and improvement in a few weeks or months has been reported in adults. Our case shows the expanding clinical spectrum of anti-GQ1b positive cranial neuropathy as early-onset AO and prolonged mydriasis more than one year.
萨卡里亚·居内什A、马拉什·根奇H、于尤尔·亚尔钦E、伊尔马兹V、萨鲁汉·迪雷斯凯内利G、卡拉B。急性眼肌麻痹和持续性瞳孔散大：扩大一名5.5岁女孩抗GQ1b阳性颅神经病变的临床谱。《土耳其儿科学杂志》2019年；61:794 - 797。无共济失调的急性眼肌麻痹（AO）是米勒-费希尔综合征（MFS）的一种非典型形式，在儿童中罕见。与MFS一样，AO患者可检测到抗GQ1b抗体。一名5.5岁女孩出现完全性眼肌麻痹、视力模糊、上睑下垂、复视和对光或近刺激无反应的瞳孔散大，意识和深腱反射保留。她没有共济失调。脑脊液（CSF）检查和头颅MRI正常。血清抗GQ1b抗体阳性。她被诊断为AO，并医嘱静脉注射免疫球蛋白（IVIG），400mg/kg/天，共5天。几周内眼肌麻痹和视力模糊有所改善。在第一年年底，瞳孔散大仍然存在，但有所改善并对近刺激有反应。约50%的MFS患者可能出现瞳孔受累，成人中报告在几周或几个月内有所改善。我们的病例显示了抗GQ1b阳性颅神经病变的临床谱扩大，表现为早发性AO和持续一年以上的瞳孔散大。
