Objective:To report the neurologic prognosis and autoimmune complications of 16 cases of childhood herpes simplex virus encephalitis.Methods:The study was conducted atŞanlıurfa Training and Research Hospital,Turkey fr...Objective:To report the neurologic prognosis and autoimmune complications of 16 cases of childhood herpes simplex virus encephalitis.Methods:The study was conducted atŞanlıurfa Training and Research Hospital,Turkey from June 2017 to August 2019.The study included 16 pediatric patients aged between 6 months and 17 years(median age 77.7 months)who were diagnosed with herpes simplex virus type 1 encephalitis by pediatric infectious disease and pediatric neurology clinics.Patients were followed using patient records,and interviews at the pediatric neurology clinic or via the telephone.Clinical and demographic data,received therapies,neurologic prognosis and complications were evaluated.Results:Patients with and without autoimmune encephalitis were compared in terms of age,sex,symptom duration before treatment,initial cerebrospinal fluid protein,glucose,red blood count and white blood count but no significant difference was found.Autoimmune complications were seen in four patients.N-methyl-D-aspartate encephalitis was observed in three patients and choreoathetosis was seen in one patient.The average follow-up period was 48.3 months.Twenty-five percent of the patients were receiving multiple antiepileptic drug(AED)treatment,43.8%were receiving single AED treatment and 31.3%were not receiving AED treatment at the end of the follow-up.Motor disability was observed in 12.5%and drug-resistant epilepsy was observed in 6.3%who had autoimmune complications.Conclusions:Seizures and movement disorders were controlled with immunotherapy and autoantibodies should be studied routinely.Treatment should be started early upon recognition of autoimmune complications through follow-up by measuring autoantibody levels and clinical examination results.Effective prevention and curative treatment modalities are needed to avoid herpes simplex virus encephalitis complications.展开更多
Background Anti-leucine-rich glioma-inactivated 1(LGI-1)autoimmune encephalitis(AE),characterized by rapid decline of memory,seizures,and neuropsychiatric abnormalities,is a rare but devastating disorder.Early diagnos...Background Anti-leucine-rich glioma-inactivated 1(LGI-1)autoimmune encephalitis(AE),characterized by rapid decline of memory,seizures,and neuropsychiatric abnormalities,is a rare but devastating disorder.Early diagnosis and treatment are essential to prevent long-term sequelae.In this report,we provide a detailed description of clinical characteristics,laboratory test results,imaging,and electroencephalography(EEG)findings,as well as treatment responses of eight patients with anti-LGI-1 AE treated at our center.Case presentation At the onset,all eight patients presented with confusion/memory deterioration,seizures(including faciobrachial dystonic seizures or other types of seizure),and behavioral changes such as hallucination,paranoia,and anxiety.Four patients were found with severe hyponatremia.Anti-LGI1 antibodies were detected in the cerebrospinal fluid and/or serum of all patients.For patients with faciobrachial dystonic seizures,no discernible scalp EEG change was detected,while EEG recording of patients experiencing other types of seizure showed focal slowing,focal epileptiform discharges,and focal onset seizures.All patients showed abnormal brain magnetic resonance imaging signals,mainly involving the mesial temporal lobe and the hippocampus.In addition,one patient also experienced fulminant cerebral edema during the acute phase of the illness.All patients received immunotherapy and anti-seizure medications and achieved good seizure control.Nevertheless,these patients continued to experience cognitive impairment during their long-term follow-ups.Conclusions The care of anti-LGI1 AE patients requires rapid evaluation,prompt initiation of immunotherapy,and long-term follow-up.The long-term presence of neurocognitive complications observed in these patients underline the importance of developing reliable biomarkers that can distinguish between different subtypes of this disease with heterogeneous clinico-electrographico-radiological features.Further research is needed to understand the molecular mechanisms underlying the heterogeneity,in order to facilitate development of more effective treatments for anti-LGI1 AE.展开更多
Autoimmune epilepsy(AE)is a general term to describe recurrent seizures that have an immune-mediated origin.It is increasingly being recognized as a cause of epilepsy due to accumulating evidence supporting an immune-...Autoimmune epilepsy(AE)is a general term to describe recurrent seizures that have an immune-mediated origin.It is increasingly being recognized as a cause of epilepsy due to accumulating evidence supporting an immune-mediated pathogenesis in patients who have shown resistance to traditional antiepileptic drugs(AEDs).The diagnosis of AE is one of the exclusions.Currently,there are no strict diagnostic guidelines for AE,and it is similarly under-recognized.The importance of early diagnosis of AE cannot be overstated,as prompt immunotherapy is important for seizure reduction.Further investigations into potential biomarkers are needed for early detection of AE and include targeted immunotherapies in combination with AEDs.The goal of this review was to provide an overview of the following biomarkers that have been associated with AE:AMPAR,LGl1,CASPR2,DPPX,GABAAR,GABABR,GFAP,GlyR,mGluR5,NMDAR,VGCC(P/Q types),amphiphysin,ANNA-1,CRMP-5,GAD65,and Ma1/Ma2 antibodies.展开更多
文摘Objective:To report the neurologic prognosis and autoimmune complications of 16 cases of childhood herpes simplex virus encephalitis.Methods:The study was conducted atŞanlıurfa Training and Research Hospital,Turkey from June 2017 to August 2019.The study included 16 pediatric patients aged between 6 months and 17 years(median age 77.7 months)who were diagnosed with herpes simplex virus type 1 encephalitis by pediatric infectious disease and pediatric neurology clinics.Patients were followed using patient records,and interviews at the pediatric neurology clinic or via the telephone.Clinical and demographic data,received therapies,neurologic prognosis and complications were evaluated.Results:Patients with and without autoimmune encephalitis were compared in terms of age,sex,symptom duration before treatment,initial cerebrospinal fluid protein,glucose,red blood count and white blood count but no significant difference was found.Autoimmune complications were seen in four patients.N-methyl-D-aspartate encephalitis was observed in three patients and choreoathetosis was seen in one patient.The average follow-up period was 48.3 months.Twenty-five percent of the patients were receiving multiple antiepileptic drug(AED)treatment,43.8%were receiving single AED treatment and 31.3%were not receiving AED treatment at the end of the follow-up.Motor disability was observed in 12.5%and drug-resistant epilepsy was observed in 6.3%who had autoimmune complications.Conclusions:Seizures and movement disorders were controlled with immunotherapy and autoantibodies should be studied routinely.Treatment should be started early upon recognition of autoimmune complications through follow-up by measuring autoantibody levels and clinical examination results.Effective prevention and curative treatment modalities are needed to avoid herpes simplex virus encephalitis complications.
文摘Background Anti-leucine-rich glioma-inactivated 1(LGI-1)autoimmune encephalitis(AE),characterized by rapid decline of memory,seizures,and neuropsychiatric abnormalities,is a rare but devastating disorder.Early diagnosis and treatment are essential to prevent long-term sequelae.In this report,we provide a detailed description of clinical characteristics,laboratory test results,imaging,and electroencephalography(EEG)findings,as well as treatment responses of eight patients with anti-LGI-1 AE treated at our center.Case presentation At the onset,all eight patients presented with confusion/memory deterioration,seizures(including faciobrachial dystonic seizures or other types of seizure),and behavioral changes such as hallucination,paranoia,and anxiety.Four patients were found with severe hyponatremia.Anti-LGI1 antibodies were detected in the cerebrospinal fluid and/or serum of all patients.For patients with faciobrachial dystonic seizures,no discernible scalp EEG change was detected,while EEG recording of patients experiencing other types of seizure showed focal slowing,focal epileptiform discharges,and focal onset seizures.All patients showed abnormal brain magnetic resonance imaging signals,mainly involving the mesial temporal lobe and the hippocampus.In addition,one patient also experienced fulminant cerebral edema during the acute phase of the illness.All patients received immunotherapy and anti-seizure medications and achieved good seizure control.Nevertheless,these patients continued to experience cognitive impairment during their long-term follow-ups.Conclusions The care of anti-LGI1 AE patients requires rapid evaluation,prompt initiation of immunotherapy,and long-term follow-up.The long-term presence of neurocognitive complications observed in these patients underline the importance of developing reliable biomarkers that can distinguish between different subtypes of this disease with heterogeneous clinico-electrographico-radiological features.Further research is needed to understand the molecular mechanisms underlying the heterogeneity,in order to facilitate development of more effective treatments for anti-LGI1 AE.
文摘Autoimmune epilepsy(AE)is a general term to describe recurrent seizures that have an immune-mediated origin.It is increasingly being recognized as a cause of epilepsy due to accumulating evidence supporting an immune-mediated pathogenesis in patients who have shown resistance to traditional antiepileptic drugs(AEDs).The diagnosis of AE is one of the exclusions.Currently,there are no strict diagnostic guidelines for AE,and it is similarly under-recognized.The importance of early diagnosis of AE cannot be overstated,as prompt immunotherapy is important for seizure reduction.Further investigations into potential biomarkers are needed for early detection of AE and include targeted immunotherapies in combination with AEDs.The goal of this review was to provide an overview of the following biomarkers that have been associated with AE:AMPAR,LGl1,CASPR2,DPPX,GABAAR,GABABR,GFAP,GlyR,mGluR5,NMDAR,VGCC(P/Q types),amphiphysin,ANNA-1,CRMP-5,GAD65,and Ma1/Ma2 antibodies.
