BACKGROUND Anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis is a rare but important complication of ovarian teratoma.Between July 2012 and December 2019,six patients with ovarian teratoma-associated anti-NMDAR en...BACKGROUND Anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis is a rare but important complication of ovarian teratoma.Between July 2012 and December 2019,six patients with ovarian teratoma-associated anti-NMDAR encephalitis were enrolled in our hospital and their clinical characteristics,treatment,and follow-up were reviewed.We also conducted a systematic literature review of ovarian teratoma related anti-NMDAR encephalitis reports between January 2014 and December 2019.AIM To better understand anti-NMDAR encephalitis through literature review and patients enrolled in our hospital.METHODS The six patients enrolled in the study were those diagnosed with anti-NMDAR encephalitis.Their history,clinical manifestations,and medications were recorded and optimum treatment provided in addition to maintaining a record of the follow-ups.In addition,we also extensively surveyed the literature and provide summarized data from 155 published cases of anti-NMDAR encephalitis from 130 case reports.PubMed and Scopus were the sources of these publications and the time period covered was 6 years ranging from January 2014 through December 2019.RESULTS The six patients enrolled for this study presented with typical symptoms resulting in a diagnosis of ovarian teratoma induced anti-NMDAR encephalitis.Appropriate interventions led to a positive outcome in all the patients,with five of six patients reporting full recovery and the sixth patient recovering with a few deficits.No death was recorded.The literature survey comprising of 155 patients cases across 130 case reports of anti-NMDAR encephalitis clearly indicated an upward trend in the reports/diagnosis in China,particularly in the surveyed time from 2014 through 2019.The majority of patients(150/155)underwent surgical intervention resulting in positive outcome.No treatment intervention was mentioned for one case while the four patients who were not surgically operated succumbed to the disease.CONCLUSION Suspected anti-NMDAR encephalitis should be quickly evaluated for anti-NMDAR antibodies since early diagnosis is important.In case of a tumor,its earliest and complete removal is recommended.Finally,early use of corticosteroids and IgG-depleting strategies(intravenous immunoglobulin or plasma exchange)may improve outcome.展开更多
BACKGROUND Anti-N-methyl-D-aspartate receptor(anti-NMDAR)encephalitis is a treatable but frequently misdiagnosed autoimmune disease.Speech dysfunction,as one of the common manifestations of anti-NMDAR encephalitis,is ...BACKGROUND Anti-N-methyl-D-aspartate receptor(anti-NMDAR)encephalitis is a treatable but frequently misdiagnosed autoimmune disease.Speech dysfunction,as one of the common manifestations of anti-NMDAR encephalitis,is usually reported as a symptom secondary to psychiatric symptoms or seizures rather than the initial symptom in a paroxysmal form.We report a case of anti-NMDAR encephalitis with paroxysmal speech disorder as a rare initial manifestation,and hope that it will contribute to the literature.CASE SUMMARY A 39-year-old man with anti-NMDAR encephalitis initially presented with paroxysmal nonfluent aphasia and was misdiagnosed with a transient ischemic attack and cerebral infarction successively.The patient subsequently presented with seizures,but no abnormalities were found on brain magnetic resonance imaging or electroencephalogram.Cerebrospinal fluid(CSF)analysis revealed mild pleocytosis and increased protein levels.Anti-NMDAR antibodies in serum and CSF were detected for a conclusive diagnosis.After immunotherapy,the patient made a full recovery.CONCLUSION This case suggests that paroxysmal speech disorder may be the presenting symptom of anti-NMDAR encephalitis in a young patient.展开更多
BACKGROUND A population-based comparative study in United States shows that the prevalence and incidence of autoimmune encephalitis are comparable to those of infectious encephalitis and its detection is increasing ov...BACKGROUND A population-based comparative study in United States shows that the prevalence and incidence of autoimmune encephalitis are comparable to those of infectious encephalitis and its detection is increasing over time.Some patients are complicated with ovarian teratoma.The younger the patient is,the less likely a tumor will be present.CASE SUMMARY This case report describes the successful treatment of anti-N-methyl-D-aspartatereceptor(NMDAR)encephalitis by early laparoscopic ovarian cystectomy and immunotherapy in a 4-year-old female child.And to the best of our knowledge,this detailed case report describes the youngest patient to date with anti-NMDAR encephalitis who underwent laparoscopic ovarian cystectomy.CONCLUSION Although the younger the patient is,the less likely a tumor will be detected,we still emphasize that all patients with suspected or confirmed anti-NMDAR encephalitis should be screened for ovarian tumors if possible.Prompt initiation of immunotherapy and tumor removal are crucial for good outcomes.展开更多
BACKGROUND Anti-N-methyl-D-aspartate receptor encephalitis(NMDARe)is capable of presenting a relapsing course and coexisting with myelin oligodendrocyte glycoprotein antibody disease,whereas it has been relatively rar...BACKGROUND Anti-N-methyl-D-aspartate receptor encephalitis(NMDARe)is capable of presenting a relapsing course and coexisting with myelin oligodendrocyte glycoprotein antibody disease,whereas it has been relatively rare.We describe a man with no history of tumor who successively developed anti-NMDARe and anti-myelin oligodendrocyte glycoprotein antibody disease.CASE SUMMARY A 29-year-old man was initially admitted with headache,fever,intermittent abnormal behavior,decreased intelligence,limb twitching and loss of consciousness on July 16,2018.On admission,examination reported no abnormality.During his presentation,he experienced aggravated symptoms,and the reexamination of cranial magnetic resonance imaging(MRI)indicated punctate abnormal signals in the left parietal lobe.External examination of cerebrospinal fluid and serum results revealed serum NMDAR antibody(Ab)(-),cerebrospinal fluid NMDAR-Ab(+)1:10 and Epstein-Barr virus capsid antigen antibody Ig G(+).Due to the imaging findings,anti-NMDARe was our primary consideration.The patient was treated with methylprednisolone and gamma globulin pulse therapy,mannitol injection dehydration to reduce intracranial pressure,sodium valproate sustained-release tablets for anti-epilepsy and olanzapine and risperidone to mitigate psychiatric symptoms.The patient was admitted to the hospital for the second time for“abnormal mental behavior and increased limb movements”on December 14,2018.Re-examination of electroencephalography and cranial MRI showed no abnormality.The results of autoimmune encephalitis antibody revealed that serum NMDAR-Ab was weakly positive and cerebrospinal fluid NMDAR-Ab was positive.Considering comprehensive recurrent anti-NMDARe,the patient was treated with propylene-hormone pulse combined with immunosuppressive agents(mycophenolate mofetil),and the symptoms were relieved.The patient was admitted for“hoarseness and double vision”for the third time on August 23,2019.Re-examination of cranial MRI showed abnormal signals in the medulla oblongata and right frontal lobe,and synoptophore examination indicated concomitant esotropia.The patient’s visual acuity further decreased,and the reexamination of cranial MRI+enhancement reported multiple scattered speckled and patchy abnormal signals in the medulla oblongata,left pons arm,left cerebellum and right midbrain,thalamus.The patient was diagnosed with an accompanying demyelinating disease.Serum antimyelin oligodendrocyte glycoprotein 1:10 and NMDAR antibody 1:10 were both positive.The patient was diagnosed with myelin oligodendrocyte glycoprotein antibody-related inflammatory demyelinating disease of the central nervous system complicated with anti-NMDARe overlap syndrome.The patient was successfully treated with methylprednisolone,gamma globulin pulse therapy and rituximab treatment.The patient remained asymptomatic and follow-up MRI scan 6 mo later showed complete removal of the lesion.CONCLUSION We emphasize the rarity of this antibody combination and suggest that these patients may require longer follow-up due to the risk of recurrence of two autoimmune disorders.展开更多
We report an atypical case of anti-N-methyl-D-aspartate receptor encephalitis(ANMDARE). A 27-year-old man diagnosed with ANMDARE received immunotherapy and had a good recovery. However, within one month, he developed ...We report an atypical case of anti-N-methyl-D-aspartate receptor encephalitis(ANMDARE). A 27-year-old man diagnosed with ANMDARE received immunotherapy and had a good recovery. However, within one month, he developed severe status epilepticus and decreased level of conscience with new hyperpyrexia and dyspnea, and was admitted to the emergency intensive care unit. Acinetobacter baumanii were found in the sputum culture; and anti-NMDAR antibodies were positive(titer: 1/80) in the cerebrospinal fluid. Repeated immunotherapy was administered with antibacterial agents, and the patient recovered except for mild psychiatric sequelae. This is the first report of ANMDARE that aggravates after acinetobacter baumannii pneumonia. Awareness and knowledge of this disorder should be extended, especially in the emergency medicine community.展开更多
ANTI-N-METHYL-D-ASPARTATE (NMDA) receptorencephalitis is a newly recognized autoimmunedisease. It is predominantly described in youngwomen with a series of symptoms, includingpersonality change, memory loss, seizures,...ANTI-N-METHYL-D-ASPARTATE (NMDA) receptorencephalitis is a newly recognized autoimmunedisease. It is predominantly described in youngwomen with a series of symptoms, includingpersonality change, memory loss, seizures, involuntarymovements, autonomic dysfunction et al.1 It is commonlyassociated with mature ovarian teratomas.2 Since its firstdenomination by Dalmau et al,1 many scientific publicationshave emerged on anti-NMDA receptor encephalitis, butonly a few focused on the anesthetic management ofpatients with this disease.3-5 Herein we reported two caseswith anti-NMDA receptor encephalitis in association withovarian teratoma and discussed the anesthetic managementand the outcomes of these patients.展开更多
Objective:An increasing number of pediatric patients are being diagnosed with anti-N-methyl-Daspartate receptor(NMDAR)encephalitis,whose treatment requires immunotherapy through nursing interventions.This study aimed ...Objective:An increasing number of pediatric patients are being diagnosed with anti-N-methyl-Daspartate receptor(NMDAR)encephalitis,whose treatment requires immunotherapy through nursing interventions.This study aimed to analyze the clinical features and long-term prognosis of pediatric anti-NMDAR encephalitis and to gather nursing experiences of immunotherapy.Methods:Seventeen children diagnosed with anti-NMDAR encephalitis were admitted to the pediatric department.They were subjected to a therapy of intravenous immunoglobulin(IVIG)accompanied with high-dose methylprednisolone(HDMP).Multidisciplinary cooperation and intensive care were used to manage them.The effects of nursing intervention and therapy were repeatedly assessed and analyzed throughout the course of treatment and recovery.Results:None of the patients manifested adverse drug reaction(ADR)during IVIG administration.At the first administration of HDMP,ADRs were promptly and efficiently treated in four patients(24%;i.e.,one case each of hyperglycosemia,hypertension,aggravated symptoms,and gastrointestinal bleed).Two patients underwent rehabilitation,and six patients received hyperbaric oxygenation during hospitalization.Nine patients with indwelling gastric tubes experienced four times of unplanned extubation.Hospital stay ranged from 11 days to 59 days,with the mean duration of 26 days.Discharge evaluation revealed that 16 patients who scored 0e2 on the modified Rankin scale presented obvious remission,and one patient who had a mRS score of 4 exhibited less improvement.The mRS scores of hospitalization,discharge,and six-month follow-up displayed statistically significant differences.Conclusions:Nursing interventions of immunotherapy ensures the security of IVIG administration.Multidisciplinary cooperation promotes remission.Our findings can serve as reference for healthcare teams.展开更多
BACKGROUND Anti-N-methyl-D-aspartate receptor(anti-NMDAR)encephalitis is a rare autoimmune disorder.The symptoms of anti-NMDAR encephalitis include behavioral problems,speech problems,psychosis,seizures,and memory def...BACKGROUND Anti-N-methyl-D-aspartate receptor(anti-NMDAR)encephalitis is a rare autoimmune disorder.The symptoms of anti-NMDAR encephalitis include behavioral problems,speech problems,psychosis,seizures,and memory deficits,among others.However,laryngospasm is rare.We present the case of a patient with anti-NMDAR antibodies and severe laryngospasms.CASE SUMMARY The patient was a 15-year-old female with normal psychomotor development.She was initially admitted to our neurological intensive care unit with seizures.She received anti-epilepsy treatment,and the seizures disappeared.However,2 wk later,she developed behavioral problems and speech impairment.Then,she developed severe laryngospasms,which were treated with intubation and a tracheotomy.Antibodies against the NMDAR were detected in the patient’s cerebrospinal fluid.Therefore,she was diagnosed with anti-NMDAR encephalitis.In addition,she received intravenously administered immunoglobulins,and methylprednisolone was administered.The patient’s symptoms gradually improved,and she was discharged from our hospital.Approximately 9 mo later,the patient could speak sentences,walk independently,and carry out activities of daily living independently.Through our case report,we highlighted laryngospasm as an uncommon presentation in patients with anti-NMDAR encephalitis.CONCLUSION Laryngospasm may be an uncommon clinical manifestation of anti-NMDAR encephalitis.展开更多
Anti-N-methyl-D-aspartate receptor-associated encephalitis(NMDARE)is a rare immune-mediated neuroinflammatory condition characterized by the rapid onset of neuropsychiatric symptoms and autonomic dysfunction.The mecha...Anti-N-methyl-D-aspartate receptor-associated encephalitis(NMDARE)is a rare immune-mediated neuroinflammatory condition characterized by the rapid onset of neuropsychiatric symptoms and autonomic dysfunction.The mechanism of pathogenesis remains incompletely understood,but is thought to be related to antibodies targeting the GluN1 subunit of the NMDA receptor with resultant downstream dysregulation of dopaminergic pathways.Young adults are most frequently affected;the median age at diagnosis is 21 years.There is a strong female predilection with a female sex predominance of 4:1.NMDARE often develops as a paraneoplastic process and is most commonly associated with ovarian teratoma.However,NMDARE has also been described in patients with small cell lung cancer,clear cell renal carcinoma,and other benign and malignant neoplasms.Diagnosis is based on correlation of the clinical presentation,electro-encephalography,laboratory studies,and imaging.Computed tomography,positron emission tomography,and magnetic resonance imaging are essential to identify an underlying tumor,exclude clinicopathologic mimics,and predict the likelihood of long-term functional impairment.Nuclear imaging may be of value for prognostication and to assess the response to therapy.Treatment may involve high-dose corticosteroids,intravenous immunoglobulin,and plasma exchange.Herein,we review the hallmark clinicopathologic features and imaging findings of this rare but potentially devastating condition and summarize diagnostic criteria,treatment regimens,and proposed pathogenetic mechanisms.展开更多
BACKGROUND Paraneoplastic limbic encephalitis(LE)is an inflammatory condition that affects the limbic system,cerebellum,and peripheral nervous system.It causes a range of symptoms including short-term memory loss,impa...BACKGROUND Paraneoplastic limbic encephalitis(LE)is an inflammatory condition that affects the limbic system,cerebellum,and peripheral nervous system.It causes a range of symptoms including short-term memory loss,impaired cognitive function,behavioral and psychological disorders,and seizures.Paraneoplastic LE can occur when an immune response is activated due to antibodies targeting gammaaminobutyric acid(GABA)B receptor(GABABR)interacting with antigens on tumor cells and the nervous system,resulting in tumors primarily as small cell lung carcinoma(SCLC).CASE SUMMARY We discuss two cases of GABABR antibody-related LE resulting from SCLC.The patients’symptoms were managed with immunotherapy but ended in premature death due to chemotherapy-related complications.CONCLUSION Paraneoplastic syndrome is a notable cause of LE.Early intravenous immunoglobulin therapy may lead to temporary remission.展开更多
BACKGROUND Anti-N-methyl-D-aspartate-receptor(NMDAR)encephalitis is a common type of autoimmune encephalitis characterized by complex clinical signs and variable imaging manifestations.The pathogenesis of the disease ...BACKGROUND Anti-N-methyl-D-aspartate-receptor(NMDAR)encephalitis is a common type of autoimmune encephalitis characterized by complex clinical signs and variable imaging manifestations.The pathogenesis of the disease is unclear.Syphilis is an infectious disease caused by Treponema pallidum that can invade the nervous and immune systems and cause systemic symptoms.There are few reports of anti-NMDAR encephalitis with syphilis,and the association between them is unknown;both diseases are related to immune system damage.We report a case of anti-NMDAR encephalitis with syphilis.CASE SUMMARY A 32-year-old man was admitted to our hospital with complaints of cognitive decline,diplopia,and walking instability during the previous 6 mo.He developed dysarthria,difficulty swallowing,and involuntary shaking of his head,neck,and limbs during the month prior to presentation.Cranial magnetic resonance imaging showed symmetrical abnormal signals in the pons,midbrain,and bilateral basal ganglia,and inflammatory demyelination was considered.The diagnosis of syphilis was confirmed based on the syphilis diagnosis test and the syphilis rapid test.He was given anti-syphilis treatment,but the above symptoms gradually worsened.Anti-NMDAR antibody was positive in cerebrospinal fluid but was negative in serum.Due to the cerebrospinal fluid findings,anti-NMDAR encephalitis was a consideration.According to the patient’s weight,he was treated with intravenous methylprednisolone 1 g QD for 5 d,with the dose gradually decreased for 6 mo,and immunoglobulin 25 g QD for 5 d;his symptoms improved after treatment.CONCLUSION This case shows that anti-NMDAR encephalitis may be combined with syphilis,which should be recognized to avoid misdiagnosis and treatment delay.展开更多
To the Editor:Anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis is a potentially lethal autoimmune disease characterized by prominent psychiatric symptoms and seizures.It usually occurs in young female patients wi...To the Editor:Anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis is a potentially lethal autoimmune disease characterized by prominent psychiatric symptoms and seizures.It usually occurs in young female patients with ovarian teratomas.[1] Here,we describe a severe case of anti-NMDAR encephalitis.The patient was originally misdiagnosed as psychosis and did not improve within the first 4 weeks of first-line immunotherapy.Although she did not continue to receive second-line immunotherapy,the patient still recovered well and had no recurrence or tumor observed during 3 years of follow-up.展开更多
Western equine encephalitis virus(WEEV)is an arbovirus that historically caused large outbreaks of encephalitis throughout the Americas.WEEV binds protocadherin 10(PCDH10)as a receptor,and highly virulent ancestral WE...Western equine encephalitis virus(WEEV)is an arbovirus that historically caused large outbreaks of encephalitis throughout the Americas.WEEV binds protocadherin 10(PCDH10)as a receptor,and highly virulent ancestral WEEV strains also bind low-density lipoprotein receptor(LDLR)-related proteins.As WEEV declined as a human pathogen in North America over the past century,isolates have lost the ability to bind mammalian receptors while still recognizing avian receptors.To explain shifts in receptor dependencies and assess the risk of WEEV re-emergence,we determined cryoelectron microscopy structures of WEEV bound to human PCDH10,avian PCDH10,and human very-low-density lipoprotein receptor(VLDLR).We show that one to three E2 glycoprotein substitutions are sufficient for a nonpathogenic strain to regain the ability to bind mammalian receptors.A soluble VLDLR fragment protects mice from lethal challenge by a virulent ancestral WEEV strain.Because WEEV recently re-emerged in South America after decades of inactivity,our findings have important implications for outbreak preparedness.展开更多
Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a rare disease with uncertain etiology and pathogenesis that affects young women. Its diagnosis can be delayed because of the nonspecific neuropsychiatric symp...Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a rare disease with uncertain etiology and pathogenesis that affects young women. Its diagnosis can be delayed because of the nonspecific neuropsychiatric symptoms in the foreground. This article describes the details of a recent complicated case of a patient with this condition which is related to an ovarian teratoma. Correct diagnostic and prompt treatment of anti-NMDA receptor encephalitis remains a serious clinical challenge due to its unspecific manifestations and varying response to treatments. The information will be of interest to clinicians working with encephalitis patients.展开更多
This study aims to discriminate between leucine-rich glioma-inactivated 1(LGI1)antibody encephalitis and gammaaminobutyric acid B(GABAB)receptor antibody encephalitis using a convolutional neural network(CNN)model.A t...This study aims to discriminate between leucine-rich glioma-inactivated 1(LGI1)antibody encephalitis and gammaaminobutyric acid B(GABAB)receptor antibody encephalitis using a convolutional neural network(CNN)model.A total of 81 patients were recruited for this study.ResNet18,VGG16,and ResNet50 were trained and tested separately using 3828 positron emission tomography image slices that contained the medial temporal lobe(MTL)or basal ganglia(BG).Leave-one-out cross-validation at the patient level was used to evaluate the CNN models.The receiver operating characteristic(ROC)curve and the area under the ROC curve(AUC)were generated to evaluate the CNN models.Based on the prediction results at slice level,a decision strategy was employed to evaluate the CNN models’performance at patient level.The ResNet18 model achieved the best performance at the slice(AUC=0.86,accuracy=80.28%)and patient levels(AUC=0.98,accuracy=96.30%).Specifically,at the slice level,73.28%(1445/1972)of image slices with GABAB receptor antibody encephalitis and 87.72%(1628/1856)of image slices with LGI1 antibody encephalitis were accurately detected.At the patient level,94.12%(16/17)of patients with GABAB receptor antibody encephalitis and 96.88%(62/64)of patients with LGI1 antibody encephalitis were accurately detected.Heatmaps of the image slices extracted using gradient-weighted class activation mapping indicated that the model focused on the MTL and BG for classification.In general,the ResNet18 model is a potential approach for discriminating between LGI1 and GABAB receptor antibody encephalitis.Metabolism in the MTL and BG is important for discriminating between these two encephalitis subtypes.展开更多
BACKGROUND It is not uncommon to develop autoimmune encephalitis and paraneoplastic neurological syndromes(PNS).4 kinds of antibody-positive autoimmune paraneoplastic limbic encephalitis(PLE)have not been reported.CAS...BACKGROUND It is not uncommon to develop autoimmune encephalitis and paraneoplastic neurological syndromes(PNS).4 kinds of antibody-positive autoimmune paraneoplastic limbic encephalitis(PLE)have not been reported.CASE SUMMARY PNS are distant effects of cancer on the nervous system,rather than syndromes in which cancer directly invades and metastasizes to the nerves and/or muscle tissues.If the limbic lobe system of the brain is involved,this will result in PLE.The detection of patients with PNS is challenging since tumors that cause paraneoplastic neurologic disorders are often asymptomatic,obscure,and thus easily misdiagnosed or missed.Currently,single-or double-antibody-positive paraneoplastic marginal encephalitis has been reported.However,no cases of three or more-antibody-positive cases have been reported.Here,we report a case of PLE that is anti-collapsing response-mediator protein-5,anti-neuronal nuclear antibody-type 1,anti-aminobutyric acid B receptor,and anti-glutamate deglutase positive,and address relevant literature to improve our understanding of the disease.CONCLUSION This article reports on the management of a case of PLE with four positive antibodies,a review of the literature,in order to raise awareness among clinicians.展开更多
Objective:To describe the clinical features of autoimmune encephalitis complicated with gastrointestinal hemorrhage.Methods:The clinical data of one patient whose initial symptom was mental abnormality were collected ...Objective:To describe the clinical features of autoimmune encephalitis complicated with gastrointestinal hemorrhage.Methods:The clinical data of one patient whose initial symptom was mental abnormality were collected and the related examinations,such as cerebrospinal fluid and magnetic resonance imaging (MRI),were improved.Results: Cerebrospinal fluid examination found that anti-α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid receptor(AMPAR)2 antibody was strongly positive,although the patient had repeated gastrointestinal hemorrhage but,after hormone combined with immunoglobulin treatment,the symptoms gradual-ly improved.Conclusion:Mental disorders are not all psychosis,and autoimmune encephalitis should not be ignored.It is very important to perform anti-AMPAR encephalitis antibody test;accurate diagnosis and timely treatment can improve the prognosis.展开更多
Anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis has been increasingly recognized in recent years.This condition may be the most common cause of antibody-mediated encephalitis worldwide.The majority of patients a...Anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis has been increasingly recognized in recent years.This condition may be the most common cause of antibody-mediated encephalitis worldwide.The majority of patients are young at the time of onset,female,and present with an acute-to-subacute onset of behavioral changes followed by seizure,abnormal movement,autonomic dysfunction,and finally hypoventilation with coma if left untreated.The immunopathogenesis of this disease may be due to antibody-mediated internalization of NMDARs from synapses,which results in the dysfunction of particular brain regions(especially the hippocampus and frontostriatal area).Compared to serum,the cerebrospinal fluid permits the more sensitive detection of anti-NMDAR antibody.Ovarian teratoma may be present in up to 40%of patients but is less frequent in children or late-onset disease(>45 years old).The severity at the time of disease onset and time to appropriate immunotherapy(high-dose steroid plus plasmapheresis or intravenous immunoglobulin)are independent factors that are associated with good outcomes.展开更多
目的探讨蛋白A免疫吸附(protein A immunoadsorption,IA)在抗N-甲基-D-天门冬氨酸受体(N‑methyl‑D‑aspartate receptor,NMDAR)脑炎患者中的应用效果及护理干预措施的意义。方法收集2021年4月至2022年8月在广东三九脑科医院神经内科行IA...目的探讨蛋白A免疫吸附(protein A immunoadsorption,IA)在抗N-甲基-D-天门冬氨酸受体(N‑methyl‑D‑aspartate receptor,NMDAR)脑炎患者中的应用效果及护理干预措施的意义。方法收集2021年4月至2022年8月在广东三九脑科医院神经内科行IA治疗的10例成年抗NMDAR脑炎患者,重点做好患者治疗前评估,确定IA治疗处方,治疗时注意各种突发事件的预防与应对,于IA治疗前一天和疗程结束后次日评估患者血清和脑脊液抗NMDAR抗体结果,于IA治疗前一天和出院前一天评估患者的症状和日常生活能力量表(Activity of Daily Living Scale,ADL)、简明精神病评定量表(Brief Psychiatric Rating Scale,BPRS)、简易精神状态评价量表(Mini Mental Status Examination,MMSE)评分变化,以及治疗期间的不良反应。采用Wilcoxon符号秩检验进行统计学分析。结果10例患者中,男6例,女4例,年龄(34.40±11.22)岁。治疗前,10例患者血清抗NMDAR抗体阳性[抗体滴度在1∶(30~320)之间],9例患者脑脊液抗NMDAR抗体阳性(抗体滴度在阴性~1∶100之间)。每例患者均行一个疗程IA治疗,共5次,隔天一次,10例患者共完成50例次治疗,其中出现1次低血压、1次皮肤过敏反应,无其他不适。与IA治疗前相比,疗程结束后次日测得10例患者血清抗NMDAR抗体滴度1∶n下降[10.0(7.5,10.0)比30.0(30.0,100.0),Z=-2.869,P=0.004],9例患者脑脊液抗NMDAR抗体滴度1∶n亦下降[10(0,10)比30(10,100),Z=-2.539,P=0.011],部分患者经IA治疗后抗体转阴。与IA治疗前相比,出院前一天患者BPRS评分下降,ADL、MMSE评分升高(均P<0.001)。结论IA治疗抗NMDAR脑炎安全有效。展开更多
基金Supported by the Shanghai Municipal Commission of Health and Family Planning,No.2017ZZ02016the Funding from National Key Research and Development Program of China,No.2021YFC2700400+1 种基金the National Natural Science Foundation of China,No.81974454the Shanghai Municipal Key Clinical Specialty,the Clinical Research Plan of SHDC,No.SHDC2020CR6009-002.
文摘BACKGROUND Anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis is a rare but important complication of ovarian teratoma.Between July 2012 and December 2019,six patients with ovarian teratoma-associated anti-NMDAR encephalitis were enrolled in our hospital and their clinical characteristics,treatment,and follow-up were reviewed.We also conducted a systematic literature review of ovarian teratoma related anti-NMDAR encephalitis reports between January 2014 and December 2019.AIM To better understand anti-NMDAR encephalitis through literature review and patients enrolled in our hospital.METHODS The six patients enrolled in the study were those diagnosed with anti-NMDAR encephalitis.Their history,clinical manifestations,and medications were recorded and optimum treatment provided in addition to maintaining a record of the follow-ups.In addition,we also extensively surveyed the literature and provide summarized data from 155 published cases of anti-NMDAR encephalitis from 130 case reports.PubMed and Scopus were the sources of these publications and the time period covered was 6 years ranging from January 2014 through December 2019.RESULTS The six patients enrolled for this study presented with typical symptoms resulting in a diagnosis of ovarian teratoma induced anti-NMDAR encephalitis.Appropriate interventions led to a positive outcome in all the patients,with five of six patients reporting full recovery and the sixth patient recovering with a few deficits.No death was recorded.The literature survey comprising of 155 patients cases across 130 case reports of anti-NMDAR encephalitis clearly indicated an upward trend in the reports/diagnosis in China,particularly in the surveyed time from 2014 through 2019.The majority of patients(150/155)underwent surgical intervention resulting in positive outcome.No treatment intervention was mentioned for one case while the four patients who were not surgically operated succumbed to the disease.CONCLUSION Suspected anti-NMDAR encephalitis should be quickly evaluated for anti-NMDAR antibodies since early diagnosis is important.In case of a tumor,its earliest and complete removal is recommended.Finally,early use of corticosteroids and IgG-depleting strategies(intravenous immunoglobulin or plasma exchange)may improve outcome.
文摘BACKGROUND Anti-N-methyl-D-aspartate receptor(anti-NMDAR)encephalitis is a treatable but frequently misdiagnosed autoimmune disease.Speech dysfunction,as one of the common manifestations of anti-NMDAR encephalitis,is usually reported as a symptom secondary to psychiatric symptoms or seizures rather than the initial symptom in a paroxysmal form.We report a case of anti-NMDAR encephalitis with paroxysmal speech disorder as a rare initial manifestation,and hope that it will contribute to the literature.CASE SUMMARY A 39-year-old man with anti-NMDAR encephalitis initially presented with paroxysmal nonfluent aphasia and was misdiagnosed with a transient ischemic attack and cerebral infarction successively.The patient subsequently presented with seizures,but no abnormalities were found on brain magnetic resonance imaging or electroencephalogram.Cerebrospinal fluid(CSF)analysis revealed mild pleocytosis and increased protein levels.Anti-NMDAR antibodies in serum and CSF were detected for a conclusive diagnosis.After immunotherapy,the patient made a full recovery.CONCLUSION This case suggests that paroxysmal speech disorder may be the presenting symptom of anti-NMDAR encephalitis in a young patient.
文摘BACKGROUND A population-based comparative study in United States shows that the prevalence and incidence of autoimmune encephalitis are comparable to those of infectious encephalitis and its detection is increasing over time.Some patients are complicated with ovarian teratoma.The younger the patient is,the less likely a tumor will be present.CASE SUMMARY This case report describes the successful treatment of anti-N-methyl-D-aspartatereceptor(NMDAR)encephalitis by early laparoscopic ovarian cystectomy and immunotherapy in a 4-year-old female child.And to the best of our knowledge,this detailed case report describes the youngest patient to date with anti-NMDAR encephalitis who underwent laparoscopic ovarian cystectomy.CONCLUSION Although the younger the patient is,the less likely a tumor will be detected,we still emphasize that all patients with suspected or confirmed anti-NMDAR encephalitis should be screened for ovarian tumors if possible.Prompt initiation of immunotherapy and tumor removal are crucial for good outcomes.
基金Supported by Health Commission of Shanxi Province Issued"Four Approval"Scientific and Technological Innovation Projects in 2020,No.2020XM38。
文摘BACKGROUND Anti-N-methyl-D-aspartate receptor encephalitis(NMDARe)is capable of presenting a relapsing course and coexisting with myelin oligodendrocyte glycoprotein antibody disease,whereas it has been relatively rare.We describe a man with no history of tumor who successively developed anti-NMDARe and anti-myelin oligodendrocyte glycoprotein antibody disease.CASE SUMMARY A 29-year-old man was initially admitted with headache,fever,intermittent abnormal behavior,decreased intelligence,limb twitching and loss of consciousness on July 16,2018.On admission,examination reported no abnormality.During his presentation,he experienced aggravated symptoms,and the reexamination of cranial magnetic resonance imaging(MRI)indicated punctate abnormal signals in the left parietal lobe.External examination of cerebrospinal fluid and serum results revealed serum NMDAR antibody(Ab)(-),cerebrospinal fluid NMDAR-Ab(+)1:10 and Epstein-Barr virus capsid antigen antibody Ig G(+).Due to the imaging findings,anti-NMDARe was our primary consideration.The patient was treated with methylprednisolone and gamma globulin pulse therapy,mannitol injection dehydration to reduce intracranial pressure,sodium valproate sustained-release tablets for anti-epilepsy and olanzapine and risperidone to mitigate psychiatric symptoms.The patient was admitted to the hospital for the second time for“abnormal mental behavior and increased limb movements”on December 14,2018.Re-examination of electroencephalography and cranial MRI showed no abnormality.The results of autoimmune encephalitis antibody revealed that serum NMDAR-Ab was weakly positive and cerebrospinal fluid NMDAR-Ab was positive.Considering comprehensive recurrent anti-NMDARe,the patient was treated with propylene-hormone pulse combined with immunosuppressive agents(mycophenolate mofetil),and the symptoms were relieved.The patient was admitted for“hoarseness and double vision”for the third time on August 23,2019.Re-examination of cranial MRI showed abnormal signals in the medulla oblongata and right frontal lobe,and synoptophore examination indicated concomitant esotropia.The patient’s visual acuity further decreased,and the reexamination of cranial MRI+enhancement reported multiple scattered speckled and patchy abnormal signals in the medulla oblongata,left pons arm,left cerebellum and right midbrain,thalamus.The patient was diagnosed with an accompanying demyelinating disease.Serum antimyelin oligodendrocyte glycoprotein 1:10 and NMDAR antibody 1:10 were both positive.The patient was diagnosed with myelin oligodendrocyte glycoprotein antibody-related inflammatory demyelinating disease of the central nervous system complicated with anti-NMDARe overlap syndrome.The patient was successfully treated with methylprednisolone,gamma globulin pulse therapy and rituximab treatment.The patient remained asymptomatic and follow-up MRI scan 6 mo later showed complete removal of the lesion.CONCLUSION We emphasize the rarity of this antibody combination and suggest that these patients may require longer follow-up due to the risk of recurrence of two autoimmune disorders.
文摘We report an atypical case of anti-N-methyl-D-aspartate receptor encephalitis(ANMDARE). A 27-year-old man diagnosed with ANMDARE received immunotherapy and had a good recovery. However, within one month, he developed severe status epilepticus and decreased level of conscience with new hyperpyrexia and dyspnea, and was admitted to the emergency intensive care unit. Acinetobacter baumanii were found in the sputum culture; and anti-NMDAR antibodies were positive(titer: 1/80) in the cerebrospinal fluid. Repeated immunotherapy was administered with antibacterial agents, and the patient recovered except for mild psychiatric sequelae. This is the first report of ANMDARE that aggravates after acinetobacter baumannii pneumonia. Awareness and knowledge of this disorder should be extended, especially in the emergency medicine community.
文摘ANTI-N-METHYL-D-ASPARTATE (NMDA) receptorencephalitis is a newly recognized autoimmunedisease. It is predominantly described in youngwomen with a series of symptoms, includingpersonality change, memory loss, seizures, involuntarymovements, autonomic dysfunction et al.1 It is commonlyassociated with mature ovarian teratomas.2 Since its firstdenomination by Dalmau et al,1 many scientific publicationshave emerged on anti-NMDA receptor encephalitis, butonly a few focused on the anesthetic management ofpatients with this disease.3-5 Herein we reported two caseswith anti-NMDA receptor encephalitis in association withovarian teratoma and discussed the anesthetic managementand the outcomes of these patients.
基金We thank the First Affiliated Hospital of the Guangxi Medical University for supplying the case data.We also thank teachers in the pediatric department for their assistance.
文摘Objective:An increasing number of pediatric patients are being diagnosed with anti-N-methyl-Daspartate receptor(NMDAR)encephalitis,whose treatment requires immunotherapy through nursing interventions.This study aimed to analyze the clinical features and long-term prognosis of pediatric anti-NMDAR encephalitis and to gather nursing experiences of immunotherapy.Methods:Seventeen children diagnosed with anti-NMDAR encephalitis were admitted to the pediatric department.They were subjected to a therapy of intravenous immunoglobulin(IVIG)accompanied with high-dose methylprednisolone(HDMP).Multidisciplinary cooperation and intensive care were used to manage them.The effects of nursing intervention and therapy were repeatedly assessed and analyzed throughout the course of treatment and recovery.Results:None of the patients manifested adverse drug reaction(ADR)during IVIG administration.At the first administration of HDMP,ADRs were promptly and efficiently treated in four patients(24%;i.e.,one case each of hyperglycosemia,hypertension,aggravated symptoms,and gastrointestinal bleed).Two patients underwent rehabilitation,and six patients received hyperbaric oxygenation during hospitalization.Nine patients with indwelling gastric tubes experienced four times of unplanned extubation.Hospital stay ranged from 11 days to 59 days,with the mean duration of 26 days.Discharge evaluation revealed that 16 patients who scored 0e2 on the modified Rankin scale presented obvious remission,and one patient who had a mRS score of 4 exhibited less improvement.The mRS scores of hospitalization,discharge,and six-month follow-up displayed statistically significant differences.Conclusions:Nursing interventions of immunotherapy ensures the security of IVIG administration.Multidisciplinary cooperation promotes remission.Our findings can serve as reference for healthcare teams.
文摘BACKGROUND Anti-N-methyl-D-aspartate receptor(anti-NMDAR)encephalitis is a rare autoimmune disorder.The symptoms of anti-NMDAR encephalitis include behavioral problems,speech problems,psychosis,seizures,and memory deficits,among others.However,laryngospasm is rare.We present the case of a patient with anti-NMDAR antibodies and severe laryngospasms.CASE SUMMARY The patient was a 15-year-old female with normal psychomotor development.She was initially admitted to our neurological intensive care unit with seizures.She received anti-epilepsy treatment,and the seizures disappeared.However,2 wk later,she developed behavioral problems and speech impairment.Then,she developed severe laryngospasms,which were treated with intubation and a tracheotomy.Antibodies against the NMDAR were detected in the patient’s cerebrospinal fluid.Therefore,she was diagnosed with anti-NMDAR encephalitis.In addition,she received intravenously administered immunoglobulins,and methylprednisolone was administered.The patient’s symptoms gradually improved,and she was discharged from our hospital.Approximately 9 mo later,the patient could speak sentences,walk independently,and carry out activities of daily living independently.Through our case report,we highlighted laryngospasm as an uncommon presentation in patients with anti-NMDAR encephalitis.CONCLUSION Laryngospasm may be an uncommon clinical manifestation of anti-NMDAR encephalitis.
文摘Anti-N-methyl-D-aspartate receptor-associated encephalitis(NMDARE)is a rare immune-mediated neuroinflammatory condition characterized by the rapid onset of neuropsychiatric symptoms and autonomic dysfunction.The mechanism of pathogenesis remains incompletely understood,but is thought to be related to antibodies targeting the GluN1 subunit of the NMDA receptor with resultant downstream dysregulation of dopaminergic pathways.Young adults are most frequently affected;the median age at diagnosis is 21 years.There is a strong female predilection with a female sex predominance of 4:1.NMDARE often develops as a paraneoplastic process and is most commonly associated with ovarian teratoma.However,NMDARE has also been described in patients with small cell lung cancer,clear cell renal carcinoma,and other benign and malignant neoplasms.Diagnosis is based on correlation of the clinical presentation,electro-encephalography,laboratory studies,and imaging.Computed tomography,positron emission tomography,and magnetic resonance imaging are essential to identify an underlying tumor,exclude clinicopathologic mimics,and predict the likelihood of long-term functional impairment.Nuclear imaging may be of value for prognostication and to assess the response to therapy.Treatment may involve high-dose corticosteroids,intravenous immunoglobulin,and plasma exchange.Herein,we review the hallmark clinicopathologic features and imaging findings of this rare but potentially devastating condition and summarize diagnostic criteria,treatment regimens,and proposed pathogenetic mechanisms.
文摘BACKGROUND Paraneoplastic limbic encephalitis(LE)is an inflammatory condition that affects the limbic system,cerebellum,and peripheral nervous system.It causes a range of symptoms including short-term memory loss,impaired cognitive function,behavioral and psychological disorders,and seizures.Paraneoplastic LE can occur when an immune response is activated due to antibodies targeting gammaaminobutyric acid(GABA)B receptor(GABABR)interacting with antigens on tumor cells and the nervous system,resulting in tumors primarily as small cell lung carcinoma(SCLC).CASE SUMMARY We discuss two cases of GABABR antibody-related LE resulting from SCLC.The patients’symptoms were managed with immunotherapy but ended in premature death due to chemotherapy-related complications.CONCLUSION Paraneoplastic syndrome is a notable cause of LE.Early intravenous immunoglobulin therapy may lead to temporary remission.
基金Supported by the National Natural Science Foundation of China,No.81571057Tianjin Science and Technology Project,No.16ZXMJSY00010
文摘BACKGROUND Anti-N-methyl-D-aspartate-receptor(NMDAR)encephalitis is a common type of autoimmune encephalitis characterized by complex clinical signs and variable imaging manifestations.The pathogenesis of the disease is unclear.Syphilis is an infectious disease caused by Treponema pallidum that can invade the nervous and immune systems and cause systemic symptoms.There are few reports of anti-NMDAR encephalitis with syphilis,and the association between them is unknown;both diseases are related to immune system damage.We report a case of anti-NMDAR encephalitis with syphilis.CASE SUMMARY A 32-year-old man was admitted to our hospital with complaints of cognitive decline,diplopia,and walking instability during the previous 6 mo.He developed dysarthria,difficulty swallowing,and involuntary shaking of his head,neck,and limbs during the month prior to presentation.Cranial magnetic resonance imaging showed symmetrical abnormal signals in the pons,midbrain,and bilateral basal ganglia,and inflammatory demyelination was considered.The diagnosis of syphilis was confirmed based on the syphilis diagnosis test and the syphilis rapid test.He was given anti-syphilis treatment,but the above symptoms gradually worsened.Anti-NMDAR antibody was positive in cerebrospinal fluid but was negative in serum.Due to the cerebrospinal fluid findings,anti-NMDAR encephalitis was a consideration.According to the patient’s weight,he was treated with intravenous methylprednisolone 1 g QD for 5 d,with the dose gradually decreased for 6 mo,and immunoglobulin 25 g QD for 5 d;his symptoms improved after treatment.CONCLUSION This case shows that anti-NMDAR encephalitis may be combined with syphilis,which should be recognized to avoid misdiagnosis and treatment delay.
文摘To the Editor:Anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis is a potentially lethal autoimmune disease characterized by prominent psychiatric symptoms and seizures.It usually occurs in young female patients with ovarian teratomas.[1] Here,we describe a severe case of anti-NMDAR encephalitis.The patient was originally misdiagnosed as psychosis and did not improve within the first 4 weeks of first-line immunotherapy.Although she did not continue to receive second-line immunotherapy,the patient still recovered well and had no recurrence or tumor observed during 3 years of follow-up.
文摘Western equine encephalitis virus(WEEV)is an arbovirus that historically caused large outbreaks of encephalitis throughout the Americas.WEEV binds protocadherin 10(PCDH10)as a receptor,and highly virulent ancestral WEEV strains also bind low-density lipoprotein receptor(LDLR)-related proteins.As WEEV declined as a human pathogen in North America over the past century,isolates have lost the ability to bind mammalian receptors while still recognizing avian receptors.To explain shifts in receptor dependencies and assess the risk of WEEV re-emergence,we determined cryoelectron microscopy structures of WEEV bound to human PCDH10,avian PCDH10,and human very-low-density lipoprotein receptor(VLDLR).We show that one to three E2 glycoprotein substitutions are sufficient for a nonpathogenic strain to regain the ability to bind mammalian receptors.A soluble VLDLR fragment protects mice from lethal challenge by a virulent ancestral WEEV strain.Because WEEV recently re-emerged in South America after decades of inactivity,our findings have important implications for outbreak preparedness.
文摘Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a rare disease with uncertain etiology and pathogenesis that affects young women. Its diagnosis can be delayed because of the nonspecific neuropsychiatric symptoms in the foreground. This article describes the details of a recent complicated case of a patient with this condition which is related to an ovarian teratoma. Correct diagnostic and prompt treatment of anti-NMDA receptor encephalitis remains a serious clinical challenge due to its unspecific manifestations and varying response to treatments. The information will be of interest to clinicians working with encephalitis patients.
基金grants from the Beijing Natural Science Foundation-Haidian Original Innovation Joint Foundation,No.L222033the National Key Research and Development Program of China“Common Disease Prevention and Control Research”Key Project,No.2022YFC2503800+2 种基金the National Natural Science Foundation of China,No.81771143the Beijing Natural Science Foundation,No.7192054and the National Key Research and Development Program of China,No.2018YFC1315201.
文摘This study aims to discriminate between leucine-rich glioma-inactivated 1(LGI1)antibody encephalitis and gammaaminobutyric acid B(GABAB)receptor antibody encephalitis using a convolutional neural network(CNN)model.A total of 81 patients were recruited for this study.ResNet18,VGG16,and ResNet50 were trained and tested separately using 3828 positron emission tomography image slices that contained the medial temporal lobe(MTL)or basal ganglia(BG).Leave-one-out cross-validation at the patient level was used to evaluate the CNN models.The receiver operating characteristic(ROC)curve and the area under the ROC curve(AUC)were generated to evaluate the CNN models.Based on the prediction results at slice level,a decision strategy was employed to evaluate the CNN models’performance at patient level.The ResNet18 model achieved the best performance at the slice(AUC=0.86,accuracy=80.28%)and patient levels(AUC=0.98,accuracy=96.30%).Specifically,at the slice level,73.28%(1445/1972)of image slices with GABAB receptor antibody encephalitis and 87.72%(1628/1856)of image slices with LGI1 antibody encephalitis were accurately detected.At the patient level,94.12%(16/17)of patients with GABAB receptor antibody encephalitis and 96.88%(62/64)of patients with LGI1 antibody encephalitis were accurately detected.Heatmaps of the image slices extracted using gradient-weighted class activation mapping indicated that the model focused on the MTL and BG for classification.In general,the ResNet18 model is a potential approach for discriminating between LGI1 and GABAB receptor antibody encephalitis.Metabolism in the MTL and BG is important for discriminating between these two encephalitis subtypes.
文摘BACKGROUND It is not uncommon to develop autoimmune encephalitis and paraneoplastic neurological syndromes(PNS).4 kinds of antibody-positive autoimmune paraneoplastic limbic encephalitis(PLE)have not been reported.CASE SUMMARY PNS are distant effects of cancer on the nervous system,rather than syndromes in which cancer directly invades and metastasizes to the nerves and/or muscle tissues.If the limbic lobe system of the brain is involved,this will result in PLE.The detection of patients with PNS is challenging since tumors that cause paraneoplastic neurologic disorders are often asymptomatic,obscure,and thus easily misdiagnosed or missed.Currently,single-or double-antibody-positive paraneoplastic marginal encephalitis has been reported.However,no cases of three or more-antibody-positive cases have been reported.Here,we report a case of PLE that is anti-collapsing response-mediator protein-5,anti-neuronal nuclear antibody-type 1,anti-aminobutyric acid B receptor,and anti-glutamate deglutase positive,and address relevant literature to improve our understanding of the disease.CONCLUSION This article reports on the management of a case of PLE with four positive antibodies,a review of the literature,in order to raise awareness among clinicians.
基金Undergraduate Innovation and Entrepreneur-ship Program Project of Hubei University of Medicine(X202210929021)Scientific research program of Hubei Provincial Department of education in 2019(Q20192103).
文摘Objective:To describe the clinical features of autoimmune encephalitis complicated with gastrointestinal hemorrhage.Methods:The clinical data of one patient whose initial symptom was mental abnormality were collected and the related examinations,such as cerebrospinal fluid and magnetic resonance imaging (MRI),were improved.Results: Cerebrospinal fluid examination found that anti-α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid receptor(AMPAR)2 antibody was strongly positive,although the patient had repeated gastrointestinal hemorrhage but,after hormone combined with immunoglobulin treatment,the symptoms gradual-ly improved.Conclusion:Mental disorders are not all psychosis,and autoimmune encephalitis should not be ignored.It is very important to perform anti-AMPAR encephalitis antibody test;accurate diagnosis and timely treatment can improve the prognosis.
文摘Anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis has been increasingly recognized in recent years.This condition may be the most common cause of antibody-mediated encephalitis worldwide.The majority of patients are young at the time of onset,female,and present with an acute-to-subacute onset of behavioral changes followed by seizure,abnormal movement,autonomic dysfunction,and finally hypoventilation with coma if left untreated.The immunopathogenesis of this disease may be due to antibody-mediated internalization of NMDARs from synapses,which results in the dysfunction of particular brain regions(especially the hippocampus and frontostriatal area).Compared to serum,the cerebrospinal fluid permits the more sensitive detection of anti-NMDAR antibody.Ovarian teratoma may be present in up to 40%of patients but is less frequent in children or late-onset disease(>45 years old).The severity at the time of disease onset and time to appropriate immunotherapy(high-dose steroid plus plasmapheresis or intravenous immunoglobulin)are independent factors that are associated with good outcomes.
文摘目的探讨蛋白A免疫吸附(protein A immunoadsorption,IA)在抗N-甲基-D-天门冬氨酸受体(N‑methyl‑D‑aspartate receptor,NMDAR)脑炎患者中的应用效果及护理干预措施的意义。方法收集2021年4月至2022年8月在广东三九脑科医院神经内科行IA治疗的10例成年抗NMDAR脑炎患者,重点做好患者治疗前评估,确定IA治疗处方,治疗时注意各种突发事件的预防与应对,于IA治疗前一天和疗程结束后次日评估患者血清和脑脊液抗NMDAR抗体结果,于IA治疗前一天和出院前一天评估患者的症状和日常生活能力量表(Activity of Daily Living Scale,ADL)、简明精神病评定量表(Brief Psychiatric Rating Scale,BPRS)、简易精神状态评价量表(Mini Mental Status Examination,MMSE)评分变化,以及治疗期间的不良反应。采用Wilcoxon符号秩检验进行统计学分析。结果10例患者中,男6例,女4例,年龄(34.40±11.22)岁。治疗前,10例患者血清抗NMDAR抗体阳性[抗体滴度在1∶(30~320)之间],9例患者脑脊液抗NMDAR抗体阳性(抗体滴度在阴性~1∶100之间)。每例患者均行一个疗程IA治疗,共5次,隔天一次,10例患者共完成50例次治疗,其中出现1次低血压、1次皮肤过敏反应,无其他不适。与IA治疗前相比,疗程结束后次日测得10例患者血清抗NMDAR抗体滴度1∶n下降[10.0(7.5,10.0)比30.0(30.0,100.0),Z=-2.869,P=0.004],9例患者脑脊液抗NMDAR抗体滴度1∶n亦下降[10(0,10)比30(10,100),Z=-2.539,P=0.011],部分患者经IA治疗后抗体转阴。与IA治疗前相比,出院前一天患者BPRS评分下降,ADL、MMSE评分升高(均P<0.001)。结论IA治疗抗NMDAR脑炎安全有效。
