Clinical analysis of four cases of anti GQ1b syndrome
10.3760/cma.j.issn.0578-1310.2017.12.016
- VernacularTitle: 抗GQ1b抗体综合征四例临床分析
- Author:
Xinying YANG
1
;
Tongli HAN
;
Weihua ZHANG
;
Shen ZHANG
;
Fang FANG
Author Information
1. Department of Neurology, Beijing Children's Hospital Affilated to Capital Medical University, Beijing 100045, China
- Publication Type:Clinical Trail
- Keywords:
Miller Fisher syndrome;
Treatment outcome;
Anti-GQ1b antibody syndrome
- From:
Chinese Journal of Pediatrics
2017;55(12):947-952
- CountryChina
- Language:Chinese
-
Abstract:
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. After treatment, condition of all patients were improved. According to the clinical manifestation, laboratory examination, and outcome after treatment, case 1 was diagnosed as anti-GQ1b antibody syndrome (Pharyngeal-Cervical-Brachial weakness overlapped with Miller Fisher syndrome), case 2 and 3 were diagnosed as anti-GQ1b antibody syndrome (Miller Fisher syndrome overlapped with Guillain Barré syndrome) and case 4 was diagnosed as anti-GQ1b antibody syndrome (acute ataxia hypersomnolence).
Conclusion:When patients with the presence of prodromic infections, monophasic course, drowsiness, ataxia, ophthalmoplegia, weakness and the symptoms/signs are relatively symmetric, anti-GQ1b antibody syndrome should be considered. Anti-GQ1b antibody has important significance for diagnosis. Most children have a good prognosis. Early correct diagnosis can avoid unnecessary examinations and guide appropriate use of immunotherapy.
