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
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
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
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
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 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
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(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 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
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...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。展开更多
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 su...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.展开更多
Background: Autoimmune encephalitis associated with antibodies against γ-aminobutyric acid B receptor (GABABR) in patients with limbic encephalitis (LE) was first described in 2010. We present a series of klan C...Background: Autoimmune encephalitis associated with antibodies against γ-aminobutyric acid B receptor (GABABR) in patients with limbic encephalitis (LE) was first described in 2010. We present a series of klan Chinese patients tbr further clinical refinement. Methods: Serum and cerebrospinal fluid (CSF) samples from patients referred to the program of encephalitis and paraneoplastic syndrome of Peking Union Medical College Hospital were tested with indirect immunofluorescence. Clinical information of patients with anti-GABABR antibody positivity was retrospectively reviewed, and descriptive statistical analysis was performed. Results: All eighteen anti-GABABR antibody-positive cases had limbic syndromes, and electroencephalogram (EEG) or neuroimaging evidence fulfilled the diagnostic criteria of LE. Four patients had additional antibodies against Hu in serum and one had anti-N-methyl-d-aspartate receptor antibody in both sera and CSF. Seventeen (17/18) patients presented with new-onset refractory seizure or status epileptics. Twelve (12/18) patients had memory deficits, 11 (11/18) patients had personality change, 7 (7/18) patients had disturbance of consciousness, and 3 (3/18) patients showed cerebellar dysfunction. One patient with LE had progressive motor and sensory polyneuropathy. Lung cancer was detected in 6 (6/18) patients. Ten (10/18) patients showed abnormality in bilateral or unilateral mediotemporal region on magnetic resonance imaging. Ten (10/18) patients had temporal lobe epileptic activity with or without general slowing on EEG. Seventeen patients received immunotherapy and 15 of them showed neurological improvement. Four patients with lung cancer died within 1-12 months due to neoplastic complications. Conclusions: Our study demonstrates that most Han Chinese patients with anti-GABABR antibody-associated LE have prominent refractory epilepsy and show neurological improvement on immunotherapy. Patients with underlying lung tumor have a relatively poor prognosis. Testing for anti-GABABR antibodies is necessary for patients with possible LE or new-onset epilepsy with unknown etiology.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
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
Encephalitis associated with antibodies to gamma-aminobutyric-acid B(GABA-B)is a subgroup of autoimmune synaptic encephalitis with typical features of limbic encephalitis and small cell lung cancer(SCLC).We report a c...Encephalitis associated with antibodies to gamma-aminobutyric-acid B(GABA-B)is a subgroup of autoimmune synaptic encephalitis with typical features of limbic encephalitis and small cell lung cancer(SCLC).We report a case of anti-GABA-B receptor encephalitis in a 57-year-old man who presented with seizures,memory loss,and abnormal behavior.He developed partially neurological responses to immunotherapy,but refused comprehensive tumor screening.The symptoms were aggravated again 4 months later.Workup showed antibodies to GABA-B receptors and tumor screening revealed SCLC.It highlights the importance of early screening of underlying tumor and anti-tumor treatment in paraneoplastic cases.展开更多
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.
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.
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.
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.
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 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。
基金This work was supported by the Joint Medical Research Project of Chongqing Science&Technology and Health Commission(2020FYYX081)High-Level Medical Reserved Personnel Training Project of Chongqing,and Natural Science Foundation of Yuzhong Direct,Chongqing(20190129).
文摘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.
文摘Background: Autoimmune encephalitis associated with antibodies against γ-aminobutyric acid B receptor (GABABR) in patients with limbic encephalitis (LE) was first described in 2010. We present a series of klan Chinese patients tbr further clinical refinement. Methods: Serum and cerebrospinal fluid (CSF) samples from patients referred to the program of encephalitis and paraneoplastic syndrome of Peking Union Medical College Hospital were tested with indirect immunofluorescence. Clinical information of patients with anti-GABABR antibody positivity was retrospectively reviewed, and descriptive statistical analysis was performed. Results: All eighteen anti-GABABR antibody-positive cases had limbic syndromes, and electroencephalogram (EEG) or neuroimaging evidence fulfilled the diagnostic criteria of LE. Four patients had additional antibodies against Hu in serum and one had anti-N-methyl-d-aspartate receptor antibody in both sera and CSF. Seventeen (17/18) patients presented with new-onset refractory seizure or status epileptics. Twelve (12/18) patients had memory deficits, 11 (11/18) patients had personality change, 7 (7/18) patients had disturbance of consciousness, and 3 (3/18) patients showed cerebellar dysfunction. One patient with LE had progressive motor and sensory polyneuropathy. Lung cancer was detected in 6 (6/18) patients. Ten (10/18) patients showed abnormality in bilateral or unilateral mediotemporal region on magnetic resonance imaging. Ten (10/18) patients had temporal lobe epileptic activity with or without general slowing on EEG. Seventeen patients received immunotherapy and 15 of them showed neurological improvement. Four patients with lung cancer died within 1-12 months due to neoplastic complications. Conclusions: Our study demonstrates that most Han Chinese patients with anti-GABABR antibody-associated LE have prominent refractory epilepsy and show neurological improvement on immunotherapy. Patients with underlying lung tumor have a relatively poor prognosis. Testing for anti-GABABR antibodies is necessary for patients with possible LE or new-onset epilepsy with unknown etiology.
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Encephalitis associated with antibodies to gamma-aminobutyric-acid B(GABA-B)is a subgroup of autoimmune synaptic encephalitis with typical features of limbic encephalitis and small cell lung cancer(SCLC).We report a case of anti-GABA-B receptor encephalitis in a 57-year-old man who presented with seizures,memory loss,and abnormal behavior.He developed partially neurological responses to immunotherapy,but refused comprehensive tumor screening.The symptoms were aggravated again 4 months later.Workup showed antibodies to GABA-B receptors and tumor screening revealed SCLC.It highlights the importance of early screening of underlying tumor and anti-tumor treatment in paraneoplastic cases.