Clinical Analysis Of Anti-NMDAR Encephalitis Combined With Double Antibody Positive

ObjectiveTo retrospectively analyze the clinical data of 7 patients with double antibody-positive anti-NMDAR encephalitis,review the existing literature and analyze the clinical characteristics of double-antibody-positive anti-NMDAR encephalitis,so as to improve neurologists’ understanding of the disease for clinical reference.MethodsClinical data including medical history,clinical manifestations,auxiliary examinations,and treatment plan were collected from 7 patients with anti-NMDAR encephalitis complicated with positive double antibodies admitted to the Department of Neurology of our hospital from September 2017 to November 2020 to retrospectively analyze the clinical characteristics of anti-NMDAR encephalitis complicated with positive double antibodies.Results1.7 double-antibody-positive anti-NMDAR encephalitis,including 1 male patient with anti-DPPX antibody positive anti-NMDAR encephalitis,the age of onset was 21 years old,and 6 patients with anti-CASPR2 antibody positive anti-NMDAR encephalitis,all of which were male.The age was 39-82 years old,and the median age of onset was 62 years old.2.Among the 7 patients,5 had acute onset and 2 had subacute onset.The average time from onset to diagnosis was 20 days.Among them,3 cases(42.9%)had no obvious prodromal symptoms,4 cases(57.1%)had prodromal symptoms such as fever,headache,dizziness,and limb weakness.4 cases(57.1%)had previous medical history,and the proportion of hypertension was relatively high.one case had herpes simplex virus encephalitis,one case had anti-NMDAR antibody encephalitis,and the remaining three cases had no special medical history.3.The clinical manifestations of 7 patients during the course of the disease were mainly epilepsy(42.9%),abnormal mental behavior(42.9%),cognitive dysfunction(14.3%),disturbance of consciousness(14.3%)and other limbic system symptoms,but also accompanied by Symptoms of involvement other than the limbic system,such as movement disorders,dizziness,headache and diarrhea.4.All 7 patients underwent thyroid function test,6 patients underwent ANA spectrum,ANA spectrum 3,ANCA spectrum test,and rheumatism.Among them,4cases(57.1%)were normal thyroid function,3 cases(42.9%)were abnormal,and 1case(14.3%)were positive for antinuclear antibodies(+),and 1 case(14.3%)was positive for anti-complement C1 q antibodies,all of which were anti-NMDAR encephalitis combined with positive anti-CASPR2 antibodies.Electrolyte examinations were performed in all 7 patients,1 patient with anti-CASPR2 antibody positive anti-NMDAR encephalitis developed hyponatremia and hypokalemia,3patients with anti-CASPR2 antibody positive anti-NMDAR encephalitis developed hyponatremia,and 2 patients combined Anti-CASPR2 antibody-positive anti-NMDAR encephalitis developed hypokalemia,and the remaining 1 case was normal.5.All 7 patients underwent cranial magnetic resonance examination.Brain MRI of anti-NMDAR encephalitis with positive anti-DPPX antibody showed atrophy of the subtentorial cerebellum and pons;one case of anti-NMDAR encephalitis with positive anti-CASPR2 antibody showed abnormal signal in the left temporal lobe;the remaining 5 cases showed scattered ischemic foci,and some were accompanied by leukoaraiosis,brain atrophy and pial vascular enhancement.Six of the seven patients underwent EEG examination.Five cases of anti-NMDAR encephalitis with anti-CASPR2 antibody positive showed diffuse slow waves in the cerebral hemispheres,and anti-NMDAR encephalitis with anti-DPPX antibody positive showed diffuse slow waves and epileptic seizure-like discharges in both cerebral hemispheres.6.7 cases of double antibody-positive anti-NMDAR encephalitis,3 of them were treated with glucocorticoid combined with gamma globulin,1 case was treated with glucocorticoid,gamma globulin combined with azathioprine triple immunotherapy,and 1 case was only given glucocorticoid Hormone therapy,5 patients were effective in immunotherapy,and the clinical symptoms improved significantly.Conclusion1.The incidence of double antibody-positive anti-NMDAR encephalitis is relatively low,and it is more common in elderly male patients.The clinical symptoms are mostly anti-NMDAR encephalitis clinical manifestations.Some patients will have variation and superposition of clinical manifestations,which may easily cause misdiagnosis or missed diagnosis.2.In anti-NMDAR encephalitis with double antibody positive,the proportion of anti-NMDAR encephalitis with anti-CASPR2 antibody positive is relatively higher,and may be secondary to intracranial infection,which leads to a longer prodromal period of the disease and atypical clinical symptoms.3.Anti-NMDAR encephalitis with double antibody-positive cranial MRI may show unilateral temporal lobe abnormal signals and brain atrophy;EEG may show diffuse slow wave and epileptiform discharge;cerebrospinal fluid examination results are complex,prone to cerebrospinal fluid Inflammation and abnormal glucose and chloride content.4.Anti-NMDAR encephalitis with double antibody positive without tumors responds well to first-line immunotherapy;the prognosis is still good;NMDAR antibody titer may not be related to the severity of clinical symptoms,and recurrence of pure anti-NMDAR encephalitis may cause superimpose antibody production.