抗GQ1b抗体综合征四例临床分析

抗GQ1b抗体综合征四例临床分析

杨欣英;韩彤立;张炜华;张珅;方方

【期刊名称】《中华儿科杂志》 【年(卷),期】2017(055)012

【摘要】Objective To investigate the clinical manifestations,laboratory findings,treatment and outcome of anti-GQ1b antibody syndrome. Method The clinical manifestations, laboratory examination, diagnosis, treatment and prognosis of (4 patients 4 male patients, from 4 to 12 years) with anti-GQ1b syndrome in Beijing Children's Hospital affiliated to Capital Medical University from 2015 to 2016 were retrospectively analyzed. Result All 4 children presented with ataxia. Case 1 showed impaired speech, ptosis and weakness of arms; case 2 and 3 had external ophthalmoplegia, weakness of limbs; case 4 presented hypersomnia, irritability and hallucinations. Serum anti-GQ1b-IgG antibody was positive in all cases.Case 1-3 received lumber puncture at the

course

of

1-2

weeks,CSF

presented

albuminocytological

dissociation,case 4 had CSF pleocytosis and increased protein level.Brain MRI of Case 1-2 were normal;Case 3 showed long T1 and T2 signal in cerebellar dentate nucleus, pons and corpus callosum; Case 4 showed long T1 and T2 signal in bilateral centrum semiovale, basal ganglia, external capsule, insula and cerebellum. Electromyograms of case 1-3 showed peripheral axonal lesion. All children were treated with IVIG.