Laryngospasm as an uncommon presentation in a patient with anti-N-methyl-D-aspartate receptor encephalitis:A case report

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.

Ovarian teratoma related anti-N-methyl-D-aspartate receptor encephalitis:A case series and review of the literature

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.

Paroxysmal speech disorder as the initial symptom in a young adult with anti-N-methyl-D-aspartate receptor encephalitis: A case report

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.

Four-year-old anti-N-methyl-D-aspartate receptor encephalitis patient with ovarian teratoma: A case report

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.

Overlapping syndrome of recurrent anti-N-methyl-D-aspartate receptor encephalitis and anti-myelin oligodendrocyte glycoprotein demyelinating diseases:A case report

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.

Anti-N-methyl-D-aspartate receptor encephalitis that aggravates after acinetobacter baumannii pneumonia:A case report

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.

Anesthetic Management of Patients with Anti-N-methyl-D-aspartate Receptor Encephalitis:A Report of Two Cases

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.

Intravenous immunoglobulin accompanied with high-dose methylprednisolone therapy for 17 children with anti-N-methyl-D-aspartate receptor encephalitis:Clinic and nursing

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.

Anti-N-methyl-D-aspartate receptor-associated encephalitis: A review of clinicopathologic hallmarks and multimodal imaging manifestations

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.

Anti-N-methyl-D-aspartate-receptor antibody encephalitis combined with syphilis:A case report

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.

Anti-N-methyl-D-aspartate receptor encephalitis in a 17-year-old female patient with 3 years of follow-up

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.

Discrimination between leucine-rich glioma-inactivated 1 antibody encephalitis and gamma-aminobutyric acid B receptor antibody encephalitis based on ResNet18

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.

Four kinds of antibody positive paraneoplastic limbic encephalitis: A rare case report

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.

Clinical features of AMPAR2 antibody-positive autoimmune encephalitis with gastrointestinal hemorrhage: a case report

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.

Anti N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis with Frustrated Diagnosis Course: A Case Report

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.

继发于单纯疱疹病毒脑炎的抗NMDAR和抗GABA_(BR)双阳性自身免疫性脑炎1例报告及文献复习

目的:分析1例单纯疱疹病毒性脑炎(HSVE)继发抗N-甲基-D-天冬氨酸受体(NMDAR)和抗γ-氨基丁酸B型受体(GABA_(BR))双阳性自身免疫性脑炎(AE)患者的临床表现及诊疗经过,以提高临床医生对该类病的认识。方法:收集1例HSVE继发抗NMDAR和抗GABA_(BR)双阳性AE患者的临床资料,对其诊断和治疗经过进行总结,并结合相关文献进行复习。结果:患者,男性,36岁,以头痛起病,随后出现肢体抽搐,并进展为意识障碍。入院后脑脊液常规生化检测异常,脑脊液单纯疱疹病毒1型(HSV-1) IgG抗体阳性,脑脊液和血清NMDAR抗体检测阳性,头部磁共振成像(MRI)检查提示右侧枕叶白质异常信号,诊断为HSVE继发抗NMDAR脑炎。数月后患者出现精神行为异常、认知障碍和睡眠障碍等症状,血清NMDAR抗体和GABA_(BR)抗体均阳性,诊断为HSVE继发抗NMDAR脑炎和抗GABA_(BR)脑炎。给予激素冲击和静脉注射免疫球蛋白(IVIG)治疗后,患者病情好转出院。随访1年,患者精神症状完全消失,遗留轻度认知功能障碍。结论:HSVE抗病毒治疗有效的恢复期患者临床症状再度恶化时,应高度怀疑继发AE的可能,应尽快完善自身免疫性抗体检测,以期早期诊断,早期治疗,以改善患者预后。

1例抗GABA_(B)R抗体相关脑炎的全程化药学监护和用药分析

1例67岁男性患者因“阵发性抽搐发作2周余”入院,诊断为抗γ-氨基丁酸B型受体(GABA_(B)R)抗体相关脑炎。入院后给予免疫治疗,治疗过程中出现癫痫持续状态、肺部感染、低蛋白血症、凝血功能异常等。临床药师结合患者病情,在免疫治疗方案的选择上给予甲泼尼龙联合静脉注射免疫球蛋白,在需要抗感染治疗时给予美罗培南(1 g q8h ivgtt);应用多烯磷脂酰胆碱护肝,给予氨溴索联合布地奈德改善肺功能,应用低分子量肝素钙预防血栓形成,对患者的营养和电解质平衡方面进行监护,给予患者全程化监护,最终患者病情好转出院。

Development from recurrent anti‑N‑methyl‑D‑aspartate receptor encephalitis with seizures as the first symptom to autoimmune‑associated epilepsy: a case report

Background Anti-N-methyl-D-aspartate receptor(anti-NMDAR)encephalitis is a novel autoimmune encephalitis(AE)first identified in 2007.It provides a new direction for clinicians when encountering unexplained symptoms such as seizures,psychotic behavioral abnormalities,speech disorders,and involuntary movements.Most patients have a good prognosis after immunotherapy,but some may experience relapses.Case presentation We report a Chinese female patient diagnosed with anti-NMDAR encephalitis.Over the past 30 years,the patient had experienced eight episodes with seizures as the first symptom,which eventually progressed to autoimmune-associated epilepsy.In the last two episodes,both serum and cerebrospinal fluid of the patient were negative for AE-related antibodies,and brain magnetic resonance imaging(MRI)revealed abnormal hyperintensity in the bilateral hippocampi.The patient’s symptoms were poorly controlled by immunotherapy but well controlled by anti-seizure medicines.Conclusions Patients with a long history of AE and multiple relapses that start with seizures may display alterations of brain structure.Physicians should pay attention to autoimmune-associated epilepsy.

28例抗体阳性自身免疫性脑炎患者的脑脊液细胞学特点分析

目的探究自身免疫性脑炎(autoimmune encephalitis,AE)患者的脑脊液(cerebral spinal fluid,CSF)细胞学特点。方法回顾性收集济宁医学院附属医院2016年1月至2019年6月收治的28例确诊AE患者的脑脊液常规、生化、细胞学、颅内压等结果,分析其特点及其与疾病严重程度的相关性。结果28例AE患者中,抗N-甲基-D-天冬氨酸受体(N-methyl-D-aspartate receptor,NMDAR)脑炎11例,抗富含亮氨酸胶质瘤失活蛋白1(leucine-rich glioma-inactivated protein 1,LGI1)抗体相关脑炎13例,抗γ-氨基丁酸B型受体(γ-aminobutyric acid type B receptor,GABA BR)抗体相关脑炎4例。CSF检查中,颅内压中位数(四分位数间距)为127.5(41.0)mmH 2O,蛋白中位数(四分位数间距)为0.48(0.15)g/L,糖和氯化物基本正常。28例中,7例患者CSF常规检查可见炎性反应;9例患者CSF细胞学检查可见炎性反应,其中6例为抗NMDAR脑炎患者,炎性反应类型均为淋巴细胞炎性反应,轻、中、重度炎性反应分别为4、3、2例,检测到激活淋巴细胞4例,浆细胞2例。CSF细胞学检查炎性细胞阳性率高于CSF常规检查炎性细胞阳性率(P=0.02),抗NMDAR脑炎患者CSF细胞学的炎性反应程度与患者入院时的改良Rankin评分(mRS)呈正相关(r=0.23,P<0.01)。结论本组AE患者CSF细胞学检查多表现为淋巴细胞炎性反应,抗NMDAR脑炎患者的CSF炎性反应阳性率最高,炎性反应程度最重。与CSF常规检查相比,CSF细胞学能够更敏感地发现AE的炎性反应,可作为诊断AE及评估疾病严重程度的重要方法。

Encephalitis associated with autoantibody binding to the anti-N-methyl-D-aspartate receptor:immunopathogenesis,mechanisms,and clinical characteristics

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.