| Objective:Anti-leucine-rich glioma inactivating protein 1(LGI1)antibody encephalitis is a central nervous system autoimmune-related disorder in which prompt diagnosis and treatment can significantly improve prognosis,and previous diagnosis has relied primarily on the detection of specific autoantibodies,which may delay diagnosis and treatment.This article reviews and analyzes the clinical data,imaging manifestations and EEG characteristics of patients with anti-LGI1 antibody encephalitis,aiming to provide a basis for the early diagnosis of clinical LGI1.Method:From July 2018 to February 2023,114 patients in the Department of Neurology of the First Hospital of Shanxi Medical University were reviewed,and autoimmune antibody tests were performed on serum and cerebrospinal fluid samples of all patients.Among them,26 patients with anti-LGI1 antibody positive were selected for analysis of clinical characteristics,imaging findings,EEG characteristics and prognosis.Outcome:1.A total of 114 patients with autoimmune encephalitis,44 cases(38.6%)positive for NMDAR antibody,26 cases(22.8%)positive for LGI1 antibody,26 cases(22.8%)positive for GABABR antibody,7 cases(6.1%)positive for Casper2 antibody,7 cases(6.1%)positive for GAD65 antibody,3 cases(2.6%)positive for AMPA2 R antibody,and 1 case(0.9%)positive for m Glu R5 antibody.2.General data results of patients with anti-LGI1 antibody encephalitis,14 cases(53.8%)in men and 12 cases(46.2%)in women;The age of onset was 19-78 years,the average age of onset was 55.65 years,5 cases(19.2%)had acute onset,14 cases(53.8%)had subacute onset,and 7 cases(26.9%)had chronic onset.3.Symptoms of anti-LGI1 antibody encephalitis patients: 9 cases(34.6%)of epilepsy;6 cases(23.1%)of mental and behavioral abnormalities;5 cases(19.2%)of cognitive impairment;dizziness in 4 cases(15.4%);1 case(3.8%)of limb weakness;Limb numbness was 1 case(3.8%).4.Clinical manifestations of patients with anti-LGI1 antibody encephalitis: mental behavior abnormalities(22/26,84.6%);seizures(20/26,76.9%);cognitive impairment(16/26,61.5%);impaired consciousness(16/26,61.5%);Autonomic dysfunction(5/26,19.2%);Speech dysfunction(3/26,11.5%).5.Seizure form in patients with anti-LGI1 antibody encephalitis: There were 14 generalized seizures(70%),which were manifested as generalized tonic-clonic seizures;There were 17 cases(85%)of focal motor seizures,including 13(65%)cases of FBDS,2cases(10%)of facial tics and 2 cases(10%)of automatisms;There were 3 cases(15%)of focal non-motor seizures,which were manifested as erect hair seizures,laughing seizures and gastric gas rise.6.Auxiliary examination results of patients with anti-LGI1 antibody encephalitis: 20cases(76.9%)of hyponatremia,15 cases of hypochloremia(57.7%),14 cases(53.8%)of elevated homocysteine,10 cases(38.5%)of hypokalemia,8 cases of abnormal liver function(30.8%),7 cases of rapid erythrocyte sedimentation rate(26.9%),6 cases of abnormal immunoglobulin(23.1%),6 cases of cytokine abnormality(23.1%),6 cases of dyslipidemia(23.1%),There were 6 cases of thyroid dysfunction,5 cases of abnormal renal function(19.2%),1 case of complement abnormality(3.8%),and 1 case of coagulation dysfunction(3.8%).7.Results of lumbar puncture in patients with anti-LGI1 antibody encephalitis: 5cases(19.2%)with abnormal intracranial pressure,12 cases(46.2%)with abnormal cerebrospinal fluid protein,10 cases(38.5%)with abnormal cerebrospinal fluid chloride,and 10 cases(38.5%)with abnormal cerebrospinal fluid glucose.8.EEG results of patients with anti-LGI1 antibody encephalitis: 19 cases(73.1%)were abnormal,of which 19(100%)patients had abnormal background activity;diffuse slow wave patients had 12 cases(63.2%);There were 10 cases(52.6%)of focal slow wave;7 cases(36.8%)had epileptiform discharge;7 clinical authors(36.8%);1 case(5.3%)of the clinical authors;There were 6 cases(31.6%)with an increase in β activity.9.Cranial magnetic resonance results of patients with anti-LGI1 antibody encephalitis:14 cases(53.8%)had abnormal signals,including medial temporal lobe(9/14,64.3%),basal ganglia(2/14,14.3%),thalamus(1/14,7.1%),paraventricular(1/14,7.1%),cerebellum(1/14,7.1%)and other lobes(1/14,7.1%).10.Patients with anti-LGI1 antibody encephalitis had APE2 and RITE2 scores,of which 23(88.5%)patients had APE2 scores ≥ 4 points,and 25(96.2%)patients had RITE2 scores ≥ 7 points.11.No tumors were found in patients with anti-LGI1 antibody encephalitis.12.Treatment and prognosis of patients with anti-LGI1 antibody encephalitis: 1patient was given intravenous methylprednisolone 1.0g/d*5 days of treatment;Twenty-one patients were treated with intravenous gamma globulin 0.4g/kg/d*5 days combined with intravenous methylprednisolone 1.0g/d*5 days;After intravenous gamma globulin 0.4g/kg/d*5 days and intravenous methylprednisolone 1.0 g/d*5 days combined with mycophenol lipid in 86.4% of patients,86.4% of patients had a good prognosis.13.Comparison results between the two groups: low cerebrospinal fluid chloride was statistically significant between normal EEG group and abnormal EEG group(P=0.03);The abnormal signal of cranial magnetic resonance was statistically significant between the FBDS group and the no FBDS group(P=0.05).Consciousness impairment and serum potassium were statistically significant between seizures and no seizures(P=<0.001,0.04).Age of onset,hyponatremia,blood sodium,blood chloride,and cerebrospinal fluid protein were statistically significant between the cognitively impaired and non-cognitive impairment groups(P=0.01,0.93,0.01,0.05,0.02).14.There were 4 cases of spasticity-like seizures,accounting for 15%,male to female ratio: 3:1,average age: 56.5 years,mainly flexion type,most patients with poor epilepsy control.Conclusion:FBDS in patients with anti-LGI1 encephalitis mostly appears in patients with abnormal brain structure,EEG findings are more non-specific,the proportion of spasmodic seizures is high,and it is refractory to treatment,which may become another characteristic seizure form of this type of disease. |
PDF Full Text Request