Clinical Analysis Of Patients With Anti-GQ1b Antibody Syndrome

Background and PurposeAnti-GQ1 b antibody syndrome is caused by the infection of microorganisms such as Campylobacter jejuni and Haemophilus influenzae,and induces the self-produced anti-GQ1 b antibody to bind to the GQ1 b antigen of the oculomotor nerve,the trochlear nerve,the abductor nerve,the muscle spindle of the limb and the brain stem.It can affect both autonomic and peripheral nervous system autoimmune diseases.Typical manifestations are extraocular muscle paralysis,ataxia,and disturbance of consciousness.It was proposed by Odaka et al.in 2001.Anti-GQ1 b antibody syndrome includes Guillain-Barré syndrome(GBS),Miller Fisher syndrome(MFS),and acute ophthalmoplegia(Acute ophthalmoplegia,AO)and Bickerstaff’s brainstem encephalitis(BBE).Subsequent studies have shown that this disease spectrum contains a wider range of diseases.A large number of studies have shown that anti-ganglioside antibodies are detected in the serum of patients with GBS lineage diseases,so ganglioside antibodies have become the most widely studied and widely used biomarkers.Gangliosides are membranous glycolipids composed of lipids and glycosyl groups,the most abundant part of which is in the nervous system.Can be divided into GT1 b,GQ1b,GD1 a,GM1 and so on..Atpresent,there have been many reports that anti-GQ1 b IgG antibodies have increased activity in GBS,MFS and their subtypes.Anti-GQ1 b antibody is a sensitive biomarker for MFS,and approximately 85% of patients with MFS have positive anti-GQ1 b IgG antibody.At present,the high sensitivity of serum ganglioside antibody detection also helps to diagnose such diseases.Because the clinical manifestations of this syndrome are complex and diverse,and the differential diagnosis is more,the clinical misdiagnosis rate is high.To this end,this article retrospectively analyzed the clinical and laboratory characteristics of 22 patients with anti-GQ1 b antibody syndrome,in order to improve the clinician’s understanding of the syndrome,early detection of early treatment of the disease.Materials and Methods1 Research objectA total of 815 serum ganglioside antibody profiles from the Neuroimmunology Laboratory of the Zhengzhou University Medical Science Research Institute from January 2013 to March 2018 were collected and analyzed for information on patients diagnosed with anti-GQ1 b antibody syndrome.A total of 22 cases.2 Experimental methodThe patient’s serum was processed strictly according to the Ou Meng blotting kit,and the clinical information of the patients diagnosed as anti-GQ1 b antibody syndrome was screened.The patient data of each patient was retrospectively analyzed.3 Follow-upThe patients who were discharged from the hospital were followed up once a month according to the date of onset,and the follow-up period was 3 months.The follow-up date was June 2018.4 Statistical methodThe data were sorted by Excel,the measurement data were expressed in the form of mean and standard deviation.,and the data were analyzed by SPSS22.0 software.The comparison between the two groups of treatment groups was performed by non-parametric Mann–Whitney U test,with P<0.05 difference,test level α=0.05.Results(1)Among the 22 patients,there were 10 males and 12 females.14 with a history of prodrome infection,including 11 cases of upper respiratory tract infection and 3cases of diarrhea.The diagnosis was as follows: GBS(n=10),MFS/BBE(n=6),MFS(n=3),BBE(n=1),BBE/GBS(n=1),AO(n=1).AO patients showed diplopia,bilateral drooping eyelids,restricted intraocular and downward activities,no ataxia and decreased sputum reflexes.Serum anti-GQ1 b antibody was positive,cerebrospinal fluid,cranial magnetic resonance and electrophysiological examination were normal.After treatment with gamma globulin and hormones,the symptoms improved.(2)Twenty-two patients underwent cerebrospinal fluid and cranial magnetic resonance imaging.There were 9 cases of cerebrospinal fluid protein-cell separation,including 6 cases of GBS(6/10,60%),1 case of MFS(1/3,33%),and 2 cases of MFS/GBS(2/6,33%).).2 cases of magnetic resonance abnormalities,1 case of BBE,MRI showed multiple abnormal signals in bilateral frontal lobe,left thalamus and brainstem;the other 1 case was BBE/GBS,and MRI showed bilateral posterior ventricle and left side of lateral ventricle Abnormal ventricular anterior horn signal.The patient’s serum was only positive for 4 patients with anti-GQ1 b antibody;18 patients with multiple antibody positive for gangliosides,including 9 cases of GBS,3 cases of MFS and MFS/GBS,1 case of AO,BBE and GBS/BBE.Of the 22 patients,3 were subjected to electromyography of the extremities,and 2 of them were demyelinating lesions of multiple peripheral nerves in the extremities.(3)Among the 22 patients,17 patients received first-line immunotherapy and 5patients received clinical symptomatic treatment.The mRS score was obtained after 1month.The first-line immunotherapy was better than the clinical symptomatic treatment,and the difference was statistically significant(P=0.039).).Five of the 17 patients were reviewed for serum anti-GQ1 b antibody after 8-12 days of treatment,and 4 of the antibodies were negative.The mRS score decreased by 2 points,2 points,1 point,1 point,and 1 patient score was not negative,drop 0 points.Seventeen(77%)patients were clinically cured within 3 months of follow-up after discharge,including4 patients with monoclonal antibody GQ1 b antibody and 13 patients withmulti-antibody-positive patients;5 patients were partially improved,,all were multi-antibody-positive patients.Of the 17 patients who had received first-line immunotherapy,13(76%)were cured within 3 months,4(24%)were unhealed,and 5(80%)were cured in 4 patients with clinical symptomatic treatment within 3months,One case of recovery(20%).Conclusions(1)Cerebrospinal fluid protein-cell separation is more common in GBS patients than in MFS patients.(2)Serum anti-gq1 b antibody with other positive ganglioside antibody may affect the course of disease and prognosis of patients,and negative conversion of anti-gq1 b antibody may serve as a good basis for clinical prognosis of patients.(3)Positive serum anti-gq1 b antibody supports the diagnosis of anti-gq1 b antibody syndrome when only ophthalmoplegia is present.