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-contactin-associated protein-like 2(CASPR2)antibody encephalitis is an autoimmune disorder characterized by the presence of antibodies against the voltage-gated potassium channel.This leads to neurolog...BACKGROUND Anti-contactin-associated protein-like 2(CASPR2)antibody encephalitis is an autoimmune disorder characterized by the presence of antibodies against the voltage-gated potassium channel.This leads to neurological symptoms,such as seizures,cognitive decline,and neuropathic pain,primarily affecting the limbic system.The prognosis of this disorder varies among individuals.CASE SUMMARY The patient,a girl aged nine years and nine months,underwent treatment for 14 to 21 d.The main clinical manifestations were vomiting and unclear consciousness,positive pathological signs,normal cranial computed tomography and magnetic resonance imaging,and abnormal electroencephalogram.The child was discharged after receiving immunoglobulin and hormone treatment.Subsequent follow-up over a period of 15 months after discharge,conducted through telephone and outpatient visits,showed no recurrence of symptoms.CONCLUSION Anti-CASPR2 antibody autoimmune encephalitis in children is rare,mainly manifested as convulsions,mental abnormalities,cognitive impairment,and neuropathic pain,among others.Timely evaluation for autoimmune encephalitis antibodies is crucial,especially in cases of recurrent central nervous system involvement in children.展开更多
BACKGROUND Asymptomatic cytomegalovirus(CMV)infection is common in children;in contrast,in children with a weakened immune system,invasive CMV can occur.This is the first case report of a severe manifestation of CMV e...BACKGROUND Asymptomatic cytomegalovirus(CMV)infection is common in children;in contrast,in children with a weakened immune system,invasive CMV can occur.This is the first case report of a severe manifestation of CMV esophagoenterocolitis in a girl diagnosed with anti-N-methyl-D-aspartate-receptor(antiNMDAR)encephalitis who received only a moderate dose of corticosteroid therapy.CASE SUMMARY A 12-year-old-Thai girl presented with acute behavioural change and headache for 6 d.Electroencephalogram and positivity for NMDAR autoantibodies were compatible with anti-NMDAR encephalitis.Hence,she received pulse methylprednisolone 10 mg/kg per day for 4 d and continued with prednisolone 1.2 mg/kg per day.On day 42 of corticosteroid therapy,she developed unremitting vomiting and diarrhoea.Endoscopy showed multiple ulcers and erythaematous mucosa along the gastrointestinal tract.Tissue CMV viral load and viral-infected cells confirmed CMV esophago-enterocolitis.Therefore,the patient received ganciclovir 5 mg/kg per dose every 12 h for 3 wk and then 5 mg/kg per dose once daily for 3 wk.Unremitting diarrhoea slowly improved from stool output 1-4 L per day to 1-2 L per day after 3 wk of treatment.Pulse methylprednisolone 20 mg/kg for 5 d was initiated and continued with prednisolone 1 mg/kg per day.After this repeated pulse methylprednisolone treatment,surprisingly,diarrhoea subsided.Immunologic work-up was performed to rule out underlying immune deficiency with unremarkable results.CONCLUSION Unremitting diarrhoea from CMV esophago-enterocolitis subsided with antiviral and methylprednisolone therapy,implying the immune and NMDAR dysregulation in anti-NMDAR encephalitis.展开更多
Neuronal surface antibody syndromes(NSAS)encompass a variety of disorders associated with“neuronal surface antibodies”.These share clinical and neuroradiological features that pose challenges related to their recogn...Neuronal surface antibody syndromes(NSAS)encompass a variety of disorders associated with“neuronal surface antibodies”.These share clinical and neuroradiological features that pose challenges related to their recognition and treatment.Recent epidemiological studies show a clear predominance for the glutamate-N-methyl-D-aspartate receptor encephalitis in both adults and pediatric population.Despite this,the overall NSAS’s incidence remains underestimated,and diagnosis persists to be not always easy to achieve.Based on current literature data,in this paper the authors propose a diagnostic pathway to approach and treat pediatric NSAS.An autoimmune etiology can be suggested through the integration of clinical,immunological,electrophysiological and neuroradiological data.On that basis,a target treatment can be started,consisting of corticosteroids and intravenous immunoglobulin or plasma exchange as a first-line immunotherapy,followed by second-line drugs including rituximab,cyclophosphamide or mycophenolate mophetil,if the case.In children a prompt diagnosis and a targeted treatment may lead to a better clinical outcome.Nevertheless further studies are required to assess the need of more tailored treatments according to long-term outcome findings and prognostic factors in different NSAS.展开更多
目的分析儿童抗接触蛋白相关蛋白-2(contactin-associated protein 2,Caspr2)抗体相关自身免疫性脑炎的临床特点、诊疗过程及预后情况,以期提高儿科医生对此病的认识。方法回顾性收集2019年6月至2022年6月山东大学附属儿童医院收治的抗C...目的分析儿童抗接触蛋白相关蛋白-2(contactin-associated protein 2,Caspr2)抗体相关自身免疫性脑炎的临床特点、诊疗过程及预后情况,以期提高儿科医生对此病的认识。方法回顾性收集2019年6月至2022年6月山东大学附属儿童医院收治的抗Caspr2抗体相关脑炎患儿4例,分析其临床资料的特征并进行随访。结果男女患儿各2例,年龄为1.3~7.0岁。4例均呈亚急性发病,首发症状表现为发热、惊厥发作4例,表现为自主神经功能障碍2例、头痛1例、精神行为异常1例;主要临床表现为意识障碍3例,言语障碍、运动障碍各2例,睡眠增多、反复头痛各1例。4例患儿头颅MRI检查和脑电图检查结果均存在异常。3例脑脊液Caspr2抗体阳性,4例血清Caspr2抗体阳性(其中1例复发时呈阳性)。3例行糖皮质激素联合免疫球蛋白治疗,1例行支持治疗,出院时病情均好转。出院后18~28个月进行随访,1例出现复发,4例改良Rankin评分0~2分。结论儿童抗Caspr2抗体相关脑炎临床表现不典型,常表现为意识障碍、发热、精神行为异常、睡眠障碍、癫痫、运动障碍、自主神经症状等。患儿对免疫治疗有较好的反应,早期诊断、早期治疗患儿预后良好。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-contactin-associated protein-like 2(CASPR2)antibody encephalitis is an autoimmune disorder characterized by the presence of antibodies against the voltage-gated potassium channel.This leads to neurological symptoms,such as seizures,cognitive decline,and neuropathic pain,primarily affecting the limbic system.The prognosis of this disorder varies among individuals.CASE SUMMARY The patient,a girl aged nine years and nine months,underwent treatment for 14 to 21 d.The main clinical manifestations were vomiting and unclear consciousness,positive pathological signs,normal cranial computed tomography and magnetic resonance imaging,and abnormal electroencephalogram.The child was discharged after receiving immunoglobulin and hormone treatment.Subsequent follow-up over a period of 15 months after discharge,conducted through telephone and outpatient visits,showed no recurrence of symptoms.CONCLUSION Anti-CASPR2 antibody autoimmune encephalitis in children is rare,mainly manifested as convulsions,mental abnormalities,cognitive impairment,and neuropathic pain,among others.Timely evaluation for autoimmune encephalitis antibodies is crucial,especially in cases of recurrent central nervous system involvement in children.
基金The Pediatric Gastroenterology and Hepatology Research Unit,ThailandGrants for Development of New Faculty Staff,Ratchadaphiseksomphot Endowment Fund,Thailand,No.RA63/012Thailand Research Fund Thailand Science Research and Innovation,Thailand,No.MRG6280190。
文摘BACKGROUND Asymptomatic cytomegalovirus(CMV)infection is common in children;in contrast,in children with a weakened immune system,invasive CMV can occur.This is the first case report of a severe manifestation of CMV esophagoenterocolitis in a girl diagnosed with anti-N-methyl-D-aspartate-receptor(antiNMDAR)encephalitis who received only a moderate dose of corticosteroid therapy.CASE SUMMARY A 12-year-old-Thai girl presented with acute behavioural change and headache for 6 d.Electroencephalogram and positivity for NMDAR autoantibodies were compatible with anti-NMDAR encephalitis.Hence,she received pulse methylprednisolone 10 mg/kg per day for 4 d and continued with prednisolone 1.2 mg/kg per day.On day 42 of corticosteroid therapy,she developed unremitting vomiting and diarrhoea.Endoscopy showed multiple ulcers and erythaematous mucosa along the gastrointestinal tract.Tissue CMV viral load and viral-infected cells confirmed CMV esophago-enterocolitis.Therefore,the patient received ganciclovir 5 mg/kg per dose every 12 h for 3 wk and then 5 mg/kg per dose once daily for 3 wk.Unremitting diarrhoea slowly improved from stool output 1-4 L per day to 1-2 L per day after 3 wk of treatment.Pulse methylprednisolone 20 mg/kg for 5 d was initiated and continued with prednisolone 1 mg/kg per day.After this repeated pulse methylprednisolone treatment,surprisingly,diarrhoea subsided.Immunologic work-up was performed to rule out underlying immune deficiency with unremarkable results.CONCLUSION Unremitting diarrhoea from CMV esophago-enterocolitis subsided with antiviral and methylprednisolone therapy,implying the immune and NMDAR dysregulation in anti-NMDAR encephalitis.
文摘Neuronal surface antibody syndromes(NSAS)encompass a variety of disorders associated with“neuronal surface antibodies”.These share clinical and neuroradiological features that pose challenges related to their recognition and treatment.Recent epidemiological studies show a clear predominance for the glutamate-N-methyl-D-aspartate receptor encephalitis in both adults and pediatric population.Despite this,the overall NSAS’s incidence remains underestimated,and diagnosis persists to be not always easy to achieve.Based on current literature data,in this paper the authors propose a diagnostic pathway to approach and treat pediatric NSAS.An autoimmune etiology can be suggested through the integration of clinical,immunological,electrophysiological and neuroradiological data.On that basis,a target treatment can be started,consisting of corticosteroids and intravenous immunoglobulin or plasma exchange as a first-line immunotherapy,followed by second-line drugs including rituximab,cyclophosphamide or mycophenolate mophetil,if the case.In children a prompt diagnosis and a targeted treatment may lead to a better clinical outcome.Nevertheless further studies are required to assess the need of more tailored treatments according to long-term outcome findings and prognostic factors in different NSAS.
文摘目的分析儿童抗接触蛋白相关蛋白-2(contactin-associated protein 2,Caspr2)抗体相关自身免疫性脑炎的临床特点、诊疗过程及预后情况,以期提高儿科医生对此病的认识。方法回顾性收集2019年6月至2022年6月山东大学附属儿童医院收治的抗Caspr2抗体相关脑炎患儿4例,分析其临床资料的特征并进行随访。结果男女患儿各2例,年龄为1.3~7.0岁。4例均呈亚急性发病,首发症状表现为发热、惊厥发作4例,表现为自主神经功能障碍2例、头痛1例、精神行为异常1例;主要临床表现为意识障碍3例,言语障碍、运动障碍各2例,睡眠增多、反复头痛各1例。4例患儿头颅MRI检查和脑电图检查结果均存在异常。3例脑脊液Caspr2抗体阳性,4例血清Caspr2抗体阳性(其中1例复发时呈阳性)。3例行糖皮质激素联合免疫球蛋白治疗,1例行支持治疗,出院时病情均好转。出院后18~28个月进行随访,1例出现复发,4例改良Rankin评分0~2分。结论儿童抗Caspr2抗体相关脑炎临床表现不典型,常表现为意识障碍、发热、精神行为异常、睡眠障碍、癫痫、运动障碍、自主神经症状等。患儿对免疫治疗有较好的反应,早期诊断、早期治疗患儿预后良好。
