| Background and ObjectiveAutoimmune encephalitis is an autoimmune disease associated with neuronal cell surface antibodies and synapsin antibodies whose targets are proteins and receptors involved in synaptic transmission and neuronal excitation.Recent years,over 10 new types of extracellular antibodies have been discovered,such as leucine-rich glioma-inactivated 1(LGI1)antibody and contactin-associated protein-like 2(CASPR2)antibody.The diseases caused by LGI1 and Caspr2 antibodies were previously attributed to antibodies against voltage-gated potassium channel antibodies,and it was subsequently discovered that the pathogenic antibodies were not directed against VGKC itself but against the protein part of VGKC complex:LGI1 and CASPR2.Therefore,diseases caused by VGKC complex related antibodies are classified into three subclasses based on different antibodies in the patient: LGI1 antibody-positive patients,Caspr2 antibody-positive patients,and patients with positive VGKC antibodies but LGI1 and Caspr2 antibodies are negative.Among them,LGI1 antibodies and Caspr2 antibodies can cause different clinical syndromes,respectively,it is not clear whether the patients with only VGKC positive have clinical relevance or not.Currently,autoimmune encephalitis associated with VGKC complex related antibodies,especially Caspr2 antibody-associated autoimmune encephalitis,is relatively rare in China.This article retrospectively analyzed the clinical data of 20 patients with VGKC complex related antibodies autoimmune encephalitis treated in the author’s hospital,combined with domestic and international literature to analyze their clinical features,and hoped to help clinicians understand the disease.Materials and MethodsThe clinical data of 20 patients with VGKC complex-related autoimmune encephalitis who were admitted to Department of Neurology,First Affiliated Hospital of Zhengzhou University from November 2015 to December 2017 were collected.All patients included in this study were required to meet the criteria for the diagnosis of autoimmune encephalitis,published in the 2016 issue of The Lancet Neurology:(1)Subacute onset,rapid progression within 3 months,suggestive of limbic involvement Symptoms such as memory impairment,epileptic seizures or psychiatric symptoms;(2)MR T2-weighted FLAIR images manifest as abnormal signals bilaterally confined to the medial temporal lobe(or brain FDG-PET suggesting medial temporal lobe hypermetabolism);(3)Increased number of cerebrospinal fluid cells(white blood cell count>5/mm3)or electroencephalographic examination revealed temporal discharges or slow waves in temporal lobe;(4)excluded other possible causes.If you do not have one of the above(1)to(3),but have positive anti-neuronal membrane,synapsin antibodies,and rule out other possible causes,you can also directly diagnose the disease as autoimmune encephalitis.Retrospectively analyze the clinical data of the 20 patients included in this study,including: gender,age,past medical history,disease duration,onset to diagnosis,presence or absence of predisposition,prodromal symptoms,first symptoms,major symptoms,and serological correlation.Indicators of detection,routine biochemical and cerebrospinal fluid antibody testing,auxiliary examination,treatment programs,and prognosis.Results1.This group of 20 patients,17 patients were males and 3 were female.The onset age of the disease is from 23 to 82 years old.The median age is 59 years old and the most frequent age group is 50 to 70 years old.2.In this group of 20 patients,6 had acute onset,and 14 had subacute onset;4 patients(20%)had prodromal symptoms,mainly analgesia,palpitation,paroxysmal dizziness,and paroxysmal bradycardia;5 patients(25%)patients had nonspecific triggers.3.In this group of 20 patients,the initial symptoms of 15 patients(75%)were the limbic encephalitis symptoms,of which 8 patients(40%)had onset of epileptic seizures and 5 patients had cognitive impairment(25%).There were 2 cases(10%)of mental and behavior disorder.There were 2 patients(10%)with faciobrachial dystonic seizures as the initial symptom,and 3 patients with hyponatremia/ myokymia/ limb weakness as the initial symptom.4.In this group of 20 patients,95% of patients had at least one of the following three symptoms:seizures,cognitive impairments,and psychiatric symptoms during the overall course of the disease.17 patients(85%)had cognitive impairments to some degree,11(55%)had epileptic seizures,11(55%)had sleep disorders,and 9(45%)had autonomic dysfunction.7 cases(35%)developed faciobrachial dystonic seizures.Seven patients(35%)experienced psychomotor abnormalities and 7 patients(35%)developed hyponatremia.1 patient(5%)experienced involuntary muscle beating and weight loss.5.In this group of 20 patients,16 patients were tested for thyroid function and their related antibodies,in which thyroid function was normal,3 cases(19%)of thyroid antibodies were abnormal.15 cases of connective tissue diseases associated antibodies were detected,and 1 case was abnormal.12 patients were tested for serum tumor markers,4 were abnormal(33%);10 patients were tested for serum virus antibodies,2 of them(20%)were abnormal;9 patients were tested for serum paraneoplastic biomarkers and 1 case(11%)was abnormal.6.In this group of 20 patients,17 patients were examined for cerebrospinal fluid and most of them showed nonspecific changes.7.In this group of 20 patients,In 6 cases,both serum and cerebrospinal fluid were examined for autoimmune encephalitis associated antibodies,in which 6 cases(100%)of serum LGI1 antibody was positive,5 cases(83%)of cerebrospinal fluid LGI1 antibody was positive;11 patients only sent in cerebrospinal fluid for the test,one case of CASPR2 antibody was positive in cerebrospinal fluid and 10 cases of LGI1 antibody in cerebrospinal fluid were positive.In 3 cases,only serum was examined,and 3 cases of serum LGI1 antibody were positive.8.In this group of 20 patients,5(26%)head MRI findings were similar to previous reports,2 cases involved basal ganglia,1 case involved right temporal and hippocampus,right basal ganglia,1 case involved bilateral temporal lobe and hippocampus and 1 case was involved in bilateral frontal lobe and temporal lobe.All of them showed T2 WI and T2 FLAIR hyperintensity;7 patients underwent contrast-enhanced MRI scan,and 1 patient showed flaky enhancement on the right basal ganglia.9.Electroencephalography was performed in all of the 20 patients,and 6 cases(30%)were abnormal.The main manifestations were non-specific slow waves.10.No tumor was found in the 20 patients in this group.Conclusion1.VGKC complex related antibodies autoimmune encephalitis is more common in middle-aged and elderly men.The peak age of onset is 50 to 70 years.2.The most common initial symptoms of VGKC complex related antibodies autoimmune encephalitis are the limbic encephalitis symptoms.The main clinical manifestations of the disease are cognitive dysfunction,epileptic seizures,psychiatric symptoms,autonomic dysfunction,sleep disturbances,myokymia,etc.Facial dystonia seizures and hyponatremia also may occur.3.Serum virology,rheumatism immune markers,and tumor markers are important for the identification of the disease.4.The routine biochemical cytological examination of CSF in patients with VGKC complex related antibodies autoimmune encephalitis shows non-specific changes,and the detection of autoimmune encephalitis-related antibodies is important for the diagnosis of the disease.5.The head MRI abnormalities in patients with VGKC complex related antibodies autoimmune encephalitis may manifest as T2 Flair hyperintense in the temporal lobe,hippocampus and basal ganglia.The EEG abnormalities manifests as non-specific diffuse slow wave.6.Patients with VGKC complex related antibodies autoimmune encephalitis are less likely to have tumors.This disease is effective for immunotherapy and may recur. |
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