| Objective:1.To investigate clinical features and treatment experience of the anti-GABABR encephalitis.2.Analyze the clinical characteristics of anti-LGI1 encephalitis and anti-GABABR encephalitis in Chinese.Then compare the characteristics of the two kinds of encephalitis.Methord:1.The clinical manifestations,laboratory and imaging data,diagnosis and treatment process of a case of anti-GABABR encephalitis in Hebei General Hospital was reviewed.2.Literature about cases report of Chinese with anti-LGI1 encephalitis and anti-GABABR encephalitis from January 2010 to January 2018 was retrieved from Pubmed,China national knowledge internet and WanFang data.Clinical information of patients with the two kind of encephalitis was retrospectively reviewed,and descriptive statistical analysis was performed.The clinical information of two kinds of encephalitis was compared and analyzed.The measurement data and numeration data was statistically analyzed with t test and?~2 test respectively.Result:1.Thirty-nine articles containing 107 cases of anti-LGI1 encephalitis and twenty-seven articles containing 111 cases of anti-GABABR encephalitis were included in the case analysis.2.Anti-LGI1encephalitis in Chinese was mainly occurred in old men meanly aged about 52 years old,the age of highest incidence was 40-49years old.The male to female ratio of patients was 1.43:1.Epileptic seizures and cognitive impairment were common initial signs of the patients.Cognitive impairment(92.5%)was the main clinical symptoms,other symptoms such as epileptic seizures(86.9%),mental behavioral abnormalities(69.2%),hyponatremia(48.6%),dyssomnia(23.4%),autonomic neuropathy(12.1%),conscious disturbance(8.4%),headache(7.5%),fever(5.6%)also occurred.There were also a few patients presented with symptoms like abnormal sensation(2.8%)and pilo-erection(0.9%).About 43%of the paients experienced faciobrachial dystonic seizures.About 4.5%of the patients presented with increased cerebrospinal fluid pressure.Routine biochemical test of cerebrospinal fluid in patients with anti-LGI1 encephalitis can be normal or mild abnormal.About 18.2%of the patients had leukocytosis,25.0%had increased protein content,8.0%had decreased chloride content and 4.5%have increased sugar content.About3.3%of the patients were accompanied by tumors and the tumers can be small cell lung cancer,thyroid tumor and fibroid.Brain MRI can be normal or showed T2/FlAIR image hyperintensity(49.1%)in temporal lobe.EEG of48.4%of the patients showed regional or diffuse slow wave and 48.4%of the patients showed epileptiform waves.Immunotherapy was the most widely used treatment.Currently,the most commonly therapy in Chinese was glucocorticoidcombinedwithintravenousimmunoglobulin,then glucocorticoid alone or intravenous immunoglobulin alone.About 97%of paitents who have recepted immunotherapy were effective.The last follow-up of the patients showed 72.4%of the patients had MRS scores of 1–2,about17.2%completely recovered,about 8.6%had MRS scores of 3–5,about 1.7%of the patients died.3.Anti-GABABR encephalitis in Chinese was occurred mainly in old men meanly aged about 56 years old,the age of highest incidence was40-49years old.The male to female ratio of cases was 2.36:1.Epileptic seizures were common initial signs of the patients.Epileptic seizures(98.2%)were also main clinical symptoms.The most common form of the seizures were generalized tonic clonic seizure(30.8%).Other symptoms such as cognitive impairment(74.8%),mental behavioral abnormalities(72.1%),conscious disturbance(46.8%),fever(17.1%),dyssomnia(16.2%),language disorder(8.1%)and autonomic neuropathy(6.5%)also occurred.There were also a few patients presented with symptoms like ataxa(4.5%),headache(3.6%),hyponatremia(1.8%),pilo-erection(0.9%),ophthalmic-myoclonu(0.9%)and supervened dysdipsia(0.9%).15.5%of the patients'cerebrospinal fluid pressure was increased.Routine biochemical test of cerebrospinal fluid in patients with anti-GABAB encephalitis can be normal or mild abnormal.About 56.4%of the patients had leukocytosis.Brain MRI can be normal or show hyperintensity in temporal lobe T2 or FlAIR image in50.5%of the patients.About 30.2%of the patients were accompanied with tumors and mainly small cell lung cancer(27.4%).EEG of 46.6%of the patients showed regional or diffuse slow wave and 32.0%of the patients showed epileptiform waves.Currently,the most commonly therapy in China was glucocorticoid combined with intravenous immunoglobulin,then glucocorticoid alone or intravenous immunoglobulin alone.The effective rate of immunotherapy was 93.2%.The last follow-up of the patients showed63.0%of the patients had MRS scores of 1–2,about 15.7%had MRS scores of3–5,about 13.9%of the patients died,about 7.4%completely recovered.4.Compared to anti-LGI1 encephalitis,the average time from onset to diagnosis of anti GABABR encephalitis was shorter.That may be attributed to the initial symptoms.Compared to anti-LGI1 encephalitis,patients with epilepsy as the first symptom were more than patients with anti GABABR encephalitis(?~2=23.333,P<0.001),but patients with cognitive impairment as the initial symptom were less(?~2=4.686,P<0.030).The symptoms of epileptic seizures may be more serious than cognitive impairment,and may be easier to attract attention and referred to hospital.5.Compared to patients with anti-LGI1 encephalitis,there were more patients with anti GABAB encephalitis sufferred from epileptic seizures(?~2=10.197,P=0.001),conscious disturbance(?~2=39.939,P<0.001)and fever(?~2=7.109,P=0.008).Compared to patients with anti GABABR encephalitis,patients with LGI1 antibody encephalitis were more easier to suffer from cognitive impairment(?~2=12.449,P<0.001)and hyponatremia(?~2=64.025,P<0.001).6.The diagnosis of the two kinds of encephalitis mainly depends on specific neuron antibody test.However,the wo kinds of encephalitis serum and cerebrospinal fluid antibody test may have negative results.The positive rate of serum specific neuron antibody(98.9%)in the patients with anti LGI1encephalitis was higher than that of cerebrospinal fluid(79.8%).The positive rate of cerebrospinal fluid specific neuron antibody(99%)in the patients with anti GABABR encephalitis was higher than the positive rate of serum antibody(95.9%).The double positive rate of serum and cerebrospinal fluid antibody test in patients with anti GABABR encephalitis was higher than that of LGI1 antibody encephalitis(?~2=11.004,P=0.001).7.The mortality of patients with anti GABABR encephalitis(13.9%)was significantly higher than that of anti-LGI1 encephalitis patients(1.7%)(?~2=6.411,P=0.011),which may be related to the combination of small cell lung cancer.Conclusion:1.In Chinese,anti-LGI1 encephalitis was frequently occurring in middle aged and old men.Most of the patients were suffered with memory impairment or epileptic seizures as the first symptom.The patients may show symptomslikecognitiveimpairment,epilepsy,mentalbehavioral abnormalities and hyponatremia etc.Cerebrospinal fluid tests can be normal or show leukocytosis,increased protein content and decreased chloride.About half of the patients'brain MRI showed high signal T2/FlAIR image in unilateral or bilateral temporal lobe and about half of the patients'EEG showed localized or diffuse slow wave.The patients responded well to immunotherapy.The effective rate of immunotherapy was 97%.For about32%of the patients showed cognitive impairment as the first symptom,the onset of the disease may be insidious,it is easier to be ignored than anti-GABABR encephalitis.Therefore,the middle-aged and old men who suffered from the symptoms of cognitive impairment such as memory loss are suggested to pay attention and go to hospital without delay.2.In Chinese,anti-GABABR encephalitis was frequently occurring in middle aged and old men.Most of the patients were suffered with epilepsy seizures as the first symptom.The patients may show symptoms like epileptic seizures,cognitive impairment,psychiatric symptoms and disturbance of consciousness etc.The cerebrospinal fluid of about more than half of the patients showed leukocytosis.About half of the patients'brain MRI showed high signal T2/FlAIR image in unilateral or bilateral temporal lobe and about half of the patients'EEG showed localized or diffuse slow wave.About 30.2%of the patients were accompanied with tumors,and most of them were small cell lung cancer.The patients responded well to immunotherapy.The effective rate of immunotherapy was 93.2%.The mortality rate is higher than that of anti-LGI1 encephalitis because many patients was combined with tumor.Early tumor detected and resection is recommended.3.In patients with anti-GABABR encephalitis,the incidence rate of epileptic seizures,conscious disturbance and fever were higher than patients with anti-LGI1 encephalitis,and the incidence rate of cognitive impairment and hyponatremia were lower than patients with anti-LGI1 encephalitis.4.The diagnosis of two kinds of encephalitis mainly depended on specific neuron antibody.But the detection of serum and cerebrospinal fluid antibody may be negative.Therefore,in order to avoid missing diagnosis,it is necessary to detect the specific neuron antibody in serum and cerebrospinal fluid at the same time. |
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