Autoimmune encephalitis with double positive anti-NMDAR and anti-GABABR secondary to herpes simplex virus encephalitis:A case report and literature review / 吉林大学学报(医学版)

Objective:To analyze the clinical presentations and diagnostic and treatment process of one patient with autoimmune encephalitis(AE)with double positive anti-N-methyl-D-aspartate receptor(NMDAR)and anti-γ-aminobutyric acid B receptor(GABABR)secondary to herpes simplex virus encephalitis(HSVE),and to improve the clinicians'awareness of this disease.Methods:The clinical data of one AE patient with double positive anti-NMDAR and anti-GABABR secondary to HSVE were collected,the diagnostic and therapeutic processes were summarized,and the relevant literatures were reviewed.Results:The patient,a 36-year-old male,developed a headache followed by limb convulsions,and progressed to disturbed consciousness.After admission,the routine biochemistry of the cerebrospinal fluid(CSF)was abnormal,and the herpes simplex virus-1(HSV-1)IgG antibody showed positive in the CSF;both CSF and serum tests for NMDAR antibodies were positive;the head magnetic resonance imaging(MRI)results showed abnormal signals in the right occipital white matter,leading to the diagnosis of HSVE secondary to anti-NMDAR encephalitis.Several months later,the patient experienced psychiatric behavior abnormalities,cognitive dysfunction,and sleep disorders,and both the serum NMDAR and GABABR antibodies showed positive results,prompting the diagnosis of HSVE secondary anti-NMDAR encephalitis and anti-GABABR encephalitis.After treatment with steroid pulse therapy and intravenous immunoglobulin(IVIG),the patient's condition was improved and the patient was discharged.At one-year follow-up,the patient's psychiatric symptoms had completely resolved,leaving mild cognitive impairment.Conclusion:If the clinical symptoms of the patients recovering from antiviral treatment for HSVE is worsened,secondary AE should be highly suspected;it is important to complete autoimmunity antibody testing as soon as possible for the early diagnosis and treatment to improve the prognosis of the patient.

The correlation between the increase of peripheral blood B cells and the recurrence of autoimmune encephalitis / 中华神经科杂志

Objective:To analyze the correlation between peripheral blood lymphocyte subsets, especially B cells, and the relapse of autoimmune encephalitis (AE).Methods:A retrospective analysis was conducted on patients with AE who were diagnosed and treated in Peking Union Medical College Hospital from January 2012 to January 2023. The clinical data including gender, age and changes in related indicators of CD19 +B cells, CD16/56 +NK cells, CD3 +T cells, CD4 +T cells, CD8 +T cells, IgG, IgA, and IgM before and after recurrence were analyzed.Binary Logistic regression analysis was applied to the study of correlation between AE recurrence and gender, age, CD19 +B cells, CD16/56 +NK cells, CD3 +T cells, CD4 +T cells, CD8 +T cells, IgG, IgA and IgM. The receiver operating characteristic (ROC) curves of the cells that affect AE recurrence (CD19 +B cells, CD16/56 +NK cells, CD3 +T cells, CD4 +T cells and CD8 +T cells) were plotted separately. Results:A total of 198 eligible AE patients were included, including 98 males and 100 females, aged (39.52±17.91) years. Among these patients, 78 cases had relapses, with a recurrence rate of 39.4%. The results of Logistic regression analysis showed that CD19 +B cells ( B=0.006, P<0.001), CD16/56 +NK cells ( B=0.004, P<0.05), CD3 +T cells ( B=-0.011, P<0.05), CD4 +T cells ( B=0.014, P<0.05) and CD8 +T cells ( B=0.010, P<0.05) were highly correlated with the relapse of AE. ROC curve analysis showed that CD19 +B cells (area under the curve: 0.833, P<0.001, critical value: 73.5/μl; sensitivity: 69.2%, specificity: 86.7%), CD3 +T cells (area under the curve: 0.784, P<0.001), CD4 +T cells (area under the curve: 0.808, P<0.001), and CD8 +T cells (area under the curve: 0.742, P<0.001) all had a certain predictive value for AE relapse. Among all the indicators, the area under the curve of CD19 +B cells was the largest, which had a higher value in predicting AE recurrence. Conclusion:The increase in peripheral blood CD19 +B cells has high predictive value for the relapse of AE.

Effect of application of anti-seizure medications on the development of chronic epilepsy after autoimmune encephalitis / 中华神经科杂志

Objective:To investigate and analyze the use and duration of anti-seizure medications (ASMs) in patients with chronic epilepsy after autoimmune encephalitis (AE), as well as the effect of ASMs use on the formation of this epilepsy to provide relevant evidence for the choice of ASMs in patients with acute seizure or chronic epilepsy after AE.Methods:A retrospective follow-up study was performed on AE patients (including patients with antibody-negative autoimmune limbic encephalitis) diagnosed in the Affiliated Brain Hospital of Nanjing Medical University from December 1, 2013 to October 31, 2022. The dates of the first seizure onset and the chronic epilepsy formation (defined as 1 year after immunotherapy) were recorded. The initial time, types and numbers of ASMs used in acute symptomatic seizure (ASS) and the maintenance time, types and numbers of ASMs in chronic epilepsy period (the continuation or the combined therapy of ASMs) were collected, respectively. A Logistic regression model was used to analyze multi-influencing factors on the formation of chronic epilepsy after AE.Results:A total of 332 patients were enrolled in this study, of whom 32.5% (108/332) with antibody-negative autoimmune limbic encephalitis. In total, 54.8% (182/332) of patients were males, and the age of onset was (40.7±19.7) years. Finally, 81.0% (269/332) of participants manifested ASS, and 57.2% (190/332) developed chronic epilepsy up to the last follow-up. The follow-up time was 1-8 years, with a median of 2 years. All patients received ASMs treatment during ASS period. Among the ASS patients, 48.0% (129/269) were prescribed monotherapy of ASMs, and 52.0% (140/269) were given the combined therapy of ASMs. Of all the patients with ASMs, 70.3% (189/269) were given early ASMs treatment (within 24 hours of the seizure onset), and 29.7% (80/269) were given delayed ASMs treatment. Subsequently, 81.0% (218/269) of the ASS patients continued the ASMs treatment (>6 months), and 19.0% (51/269) stopped use of ASMs. In the chronic epilepsy stage, 79.5% (151/190) of thee epilepsy patients continued ASMs, of whom 37.1% (56/151) were treated with monotherapy, and 62.9% (95/151) were treated with combined therapy. The incidence of chronic epilepsy was 81.3% (65/80) in the delayed ASMs treatment group, higher than the 66.1% (125/189) in the early ASMs treatment group,with statistically significant difference (χ 2=6.189, P=0.013). There were no statistically significant differences in the ASMs types and whether combined therapy of ASMs was used between chronic epilepsy group and non-chronic epilepsy group. The Logistic regression model showed that delayed ASMs treatment ( OR=2.306,95% CI 1.032-6.387, P=0.018), positive anti-neuronal intracellular antibodies ( OR=2.626,95% CI 1.536-9.531, P=0.004,compared with anti- neuronal surface antibodies), abnormal brain magnetic resonance imaging ( OR=9.883,95% CI 3.608-27.071, P<0.001), elevated cerebrospinal fluid protein ( OR=2.874,95% CI 1.115-7.409, P=0.029), and abnormal electroencephalogram ( OR=9.287,95% CI 3.767-22.896, P<0.001) were independent risk factors for chronic epilepsy after AE. Conclusions:The development of chronic epilepsy after AE is associated with the occurrence of ASS and the delayed use of ASMs, but the type of ASMs or whether the combined ASMs therapy is used is not associated with the formation of chronic epilepsy after AE. It is concluded that early ASMs treatment for the AE patients with ASS may reduce the incidence of chronic epilepsy. For AE patients with ASS who have undergone early standardized treatment, long-term, combined ASMs treatment may not be necessary.

Encefalitis autoinmune seronegativa asociada a síndrome de Sjögren primario / Autoimmune seronegative encephalitis associated to primary Sjögren's disease

La encefalitis autoinmune es un trastorno inmunomediado que compromete distintos territorios del parénquima cerebral, involucrando frecuentemente la materia gris profunda o la corteza, con o sin compromiso de la materia blanca, meninges o médula espinal. Se asocia frecuentemente con enfermedades autoinmunes o paraneoplásicas, y constituye un reto diagnóstico. Reportamos el caso de una mujer de 55 años con antecedente de síndrome de Sjögren que consultó a Emergencias por cefalea y confusión. El líquido cefalorraquídeo (LCR) presentaba leucocitosis con neutrofilia. En la resonancia magnética nuclear (RMN) cerebral se evidenciaron múltiples imágenes de comportamiento restrictivo, de señal hiperintensa en T2 y FLAIR, a predominio córtico-subcortical a nivel occipital bilateral, hemisferio cerebeloso derecho y parietal derecho. Se descartaron infecciones y neoplasias. El panel de anticuerpos para encefalitis autoinmune aquaporina-4 y anti-MOG en LCR fue negativo. Recibió metilprednisolona endovenosa con mejoría progresiva de los síntomas.

Autoimmune encephalitis is an immune-mediated disorder that affects different areas of the brain parenchyma, often involving deep gray matter or the cortex, with or without involvement of white matter, meninges, or spinal cord. It is frequently associated with autoimmune or paraneoplastic diseases and is a diagnostic challenge. We report the case of a 55-year-old woman with history of Sjögren's syndrome who presented to the emergency department with headache and episodes of confusion. Cerebrospinal fluid (CSF) analysis showed leukocytosis with neutrophilia. Brain MRI revealed multiple restricted diffusion lesions with hyperintense signal on T2 and FLAIR sequences, predominantly in the bilateral occipital region, right cerebellar hemisphere, and right parietal region. Infections and neoplasms were ruled out. The panel of antibodies for autoimmune encephalitis, including Aquaporin-4 and anti-MOG in CSF, was negative. She received intravenous methylprednisolone, leading to symptom improvement.

Secuelas cognitivas en encefalitis inmunomediadas: cohorte de pacientes en Argentina / Cognitive sequelae in immunomediated encephalitis: cohort of patients in Argentina

Resumen Introducción: Las encefalitis inmunomediadas son un desorden neurológico de origen autoinmune. Actual mente es escasa la descripción de las secuelas cognitivas crónicas. El objetivo del presente trabajo fue caracterizar la secuela cognitiva de diferentes tipos de encefalitis inmunomediadas en una cohorte de un centro único de Argentina. Métodos: Estudio prospectivo, observacional, trans versal, de pacientes en seguimiento en un hospital de la Ciudad de Buenos Aires, con diagnóstico de encefalitis inmunomediada probable y definitiva. Se evaluaron variables epidemiológicas, clínicas, paraclínicas y tra tamiento. Se determinó la secuela cognitiva a través de una evaluación neurocognitiva realizada a partir del año de la presentación clínica. Resultados: Fueron incluidos 15 pacientes, todos con resultado disminuido en al menos un test. La memoria fue el dominio más afectado. Aquellos que se encon traban bajo tratamiento inmunosupresor al momento de evaluarse presentaron menores resultados en el aprendizaje seriado (media -2.94; desvío estándar 1.54) versus los que se encontraban sin tratamiento (media -1.18; desvío estándar 1.40; p = 0.05) y en la prueba de reconocimiento (media -10.34; desvío estándar 8.02) ver sus sin tratamiento (media -1.39; desvío estándar 2.21; p = 0.003). Los pacientes con estatus epiléptico tuvieron resultados deficitarios en la prueba de reconocimiento (media -7.2; desvío estándar 7.91) en comparación a los que no lo tenían (media -1.47; desvío estándar 2.34; p = 0.05). Conclusión: Nuestros resultados demuestran que, a pesar del curso monofásico de la enfermedad, todos los pacientes presentan daño cognitivo persistente más allá del año del inicio del cuadro. Estudios prospectivos de mayor envergadura serían necesarios para confirmar nuestros hallazgos.

Abstract Introduction: Autoimmune encephalitis represents a group of immune-mediated neurological disorders. At present, the description of the chronic cognitive sequela is scarce. The objective of this study was to characterize the cognitive after effects of different types of autoimmune encephalitis in a cohort from a single center in Argentina. Methods: Prospective, observational, cross-sectional study of patients under follow-up at a hospital in Buenos Aires city, with a diagnosis of probable and definitive immune-mediated encephalitis. Epidemiological, clini cal, paraclinical and treatment related variables were evaluated. Cognitive sequela was determined through a neurocognitive evaluation performed at least a year after the clinical presentation. Results: Fifteen patients were included. All had di minished results in at least one test. Memory was the most affected domain. Patients who were under im munosuppressive treatment at the time of evaluation presented lower results in serial learning (mean -2.94; standard deviation 1.54) versus those who weren't under treatment (mean -1.18; standard deviation 1.40; p = 0.05). The same pattern was observed on the recognition test of treatment group (mean -10.34; standard deviation 8.02) versus treatment-free group (mean -1.39; standard deviation 2.21; p =0.003). Patients with status epilepticus had poorer results in the recognition test (mean -7.2; standard deviation 7.91) compared to those without it (mean -1.47; standard deviation 2.34; p = 0.05). Conclusion: Our results show that, despite the mo nophasic course of this disease, all patients had persis tent cognitive damage beyond the year of onset. Larger prospective studies are required to confirm our findings.

DIAGNOSTIC DILEMMA IN TYPHOID ENCEPHALOPATHY

Using the immune system to its advantage, Salmonella Typhi initially invades the gut followed by the reticuloendothelial system and finally the nervous system, involvement of which usually occurs around the second week of fever. In developing countries, delayed diagnosis is predominantly due to hesitation in seeking treatment. Our subject presented with fever since one week, altered mentation, headache and neck pain; she was diagnosed with enteric fever. Although her neurological abnormality could be a complication of the infection, it appeared when she became afebrile- hence we evaluated her for autoimmune conditions. Positive results hinted at autoimmune encephalitis triggered by the infection; further studies were inconclusive. Association of enteric fever with autoimmune encephalitis has not been reported. Three months later, presence of antinuclear antibodies (ANA) was rechecked- a negative report led to a retrospective diagnosis of transient ANA positivity in a nonautoimmune inflammatory disease, the case in point being enteric fever.

Anti-CASPR2 antibody-associated autoimmune encephalitis： a case report / 四川精神卫生

This paper elaborated a case of an elderly patient with anti-contactin-associated protein-like 2 （CASPR2） antibody-associated autoimmune encephalitis. It illustrated the common manifestations and diagnostic process of anti-CASPR2 antibody-associated encephalitis， and the analysis of clinical symptoms and ancillary findings had contributed to gaining insight into the diagnosis of autoimmune encephalitis in elderly patients characterized by distinct psycho-behavioral abnormalities and cognitive decline， as well as suggesting the possibility that autoimmune encephalitis in elderly may exacerbate the development of cerebrovascular disease.

Clinical features of leucine-rich glioma-inactivated 1 antibody-positive autoimmune encephalitis / 中风与神经疾病杂志

@#Objective To investigate the clinical manifestations, imaging features, treatment, and prognosis of autoimmune encephalitis （AE） with positive leucine-rich glioma-inactivated 1 （LGI1） antibody. Methods A retrospective analysis was performed for the clinical data of 11 patients with LGI1 antibody-positive AE who were admitted to Department of Neurology, The Sixth Medical Center of PLA General Hospital, from 2018 to 2022. Results Among these 11 patients, there were 10 patients with epilepsy, 8 patients with cognitive impairment, 6 patients with mental and behavioral disorders, 5 patients with sleep disorders, 1 patient with speech and language impairment, 1 patient with involuntary limb movements, 1 patient with dizziness, and 7 patients with hyponatremia. All 11 patients tested positive for LGI1 antibody, and 8 patients tested positive in serum and cerebrospinal fluid; 1 patient was also positive for contactin-associated protein-like 2 antibody, and 3 patients were positive for a single antibody in serum. Lung CT showed that 1 patient had space-occupying lesion, cranial magnetic resonance imaging showed abnormalities in 6 patients, and positron emission tomography/computed tomography showed abnormalities in 3 patients. There were 7 patients with electroencephalographic abnormalities. All 11 patients had improvements in symptoms after immunotherapy. Five patients were followed up, and 6 were lost to follow-up. Conclusion The main manifestations of LGI antibody encephalitis include seizure, faciobrachial dystonic seizures, cognitive impairment, and mental and behavioral disorders accompanied by hyponatremia. The titer of LGI1 antibody in serum is more sensitive than that in cerebrospinal fluid, and a few patients may have multiple positive autoantibodies. Immunotherapy is an effective treatment method for LGI1 antibody-positive AE.

Recent advance in anti-neurexin-3α antibody-associated encephalitis / 中华神经医学杂志

Neurexin-3α, discovered in 2016, is a new type of autoimmune encephalitis antibody. Anti-neurexin-3α antibody-associated encephalitis is generally associated with prodromal symptoms or mood changes, having main clinical manifestations as seizures, memory disorders, confusion or loss of consciousness, central ventilation insufficiency, abnormal behavior, and speech disorders. This paper reviews the relevant research progress at home and abroad about pathogenesis, diagnosis and differential diagnosis, treatment and prognosis of anti-neurexin-3α antibody-associated encephalitis, so as to expand the understanding of clinicians for this disease.

Clinical characteristics of patients with autoimmune encephalitis combined with blood-brain barrier damage and their response to immunotherapy / 中华神经医学杂志

Objective:To analyze the relations of blood-brain barrier (BBB) integrity with clinical features and their response to immunotherapy in patients with autoimmune encephalitis (AE).Methods:One hundred and forty-seven AE patients confirmed in Department of Neurology, First Affiliated Hospital of Zhengzhou University from August 2015 to December 2021 were chosen; their clinical and laboratory data were collected retrospectively. In accordance with cerebrospinal fluid (CSF) albumin/serum albumin (QAlb) value of 7, patients were classified into normal BBB group ( n=101, QAlb value ≤7.00) and damaged BBB group ( n=46, QAlb value >7.00). Modified Rankin Scale (mRS) and Clinical Assessment Scale for Autoimmune Encephalitis (CASE) were used to evaluate the severity on admission and 30 d after first-line immunotherapy; and good immunotherapy responsiveness was defined as CASE or mRS scores 30 d after first-line immunotherapy lower than those at admission. Differences of general data, clinical manifestations, CSF and peripheral blood biochemical results, and response to immunotherapy between the two groups were compared and analyzed. Results:The damaged BBB group had significantly higher proportions of patients with decreased consciousness level (58.7% vs. 37.6%), increased CSF protein, increased immunoglobulin G (IgG) and increased 24 h intramural IgG synthesis rate, and significantly higher fibrinogen in peripheral blood than normal BBB group ( P<0.05). On 30 th d of treatment, mRS scores of patients in damaged BBB group were significantly higher than those of patients in normal BBB group ( P<0.05), and the differences of CASE scores and mRS scores before and after treatment in damaged BBB group were significantly lower than those in normal BBB group (0.50±0.46 vs. 3.24±2.93, 0.70±0.62 vs. 1.15±1.04, P<0.05). In damaged BBB group, good immunotherapy response rate in patients receiving single immunotherapy was significantly lower than that in patients receiving two or three combinations of first-line immunotherapy ( P<0.05). Conclusion:BBB integrity is closely related to clinical characteristics and response to immunotherapy of AE; combined first-line immunotherapy is recommended for AE patients with BBB injury.

Characteristics of acute symptomatic seizures and autoimmune associated epilepsy secondary to autoimmune encephalitis / 中华神经医学杂志

Autoimmune encephalitis (AE) is closely related to epileptic seizure, which is a common clinical manifestation or even the only symptom at acute phase of AE. Most patients do not develop seizures after treatment at acute or subacute stages, and these patients are classified as acute symptomatic seizures secondary to AE (ASSAE). Only a minority of patients will eventually develop autoimmune associated epilepsy (AAE). At present, no unified standard for clinical diagnosis is noted between ASSAE and AAE, but some differences exist in definition, pathogenesis, diagnosis and treatment. This paper summarizes the similarities and differences between ASSAE and AAE in the above aspects, aiming at providing help for clinicians to differentiate the diagnosis of the two diseases.

Recent advance in movement disorder of anti- N-methyl- D-aspartate receptor encephalitis / 中华神经医学杂志

Anti- N-methyl- D-aspartate receptor (NMDAR) encephalitis is an autoimmune disease of the central nervous system mediated by NMDAR antibodies. Movement disorder is one of the clinical features of anti-NMDAR encephalitis, with various manifestations such as orofacial involuntary movements and limbs stereotypies, which can indicate the disease diagnosis. Currently, the clinical understanding and attention to movement disorder are insufficient, with few treatment strategies. This article reviews the research progress on characteristics and treatment methods of movement disorder of anti-NMDAR encephalitis, aiming to further effectively recognize and treat movement disorder.

Anticuerpos Anti-Titina en Encefalitis Autoinmune: ¿Casualidad o Causalidad? A propósito de un caso

Las encefalitis autoinmunes son una condición emergente, caracterizada por la aparición repentina de síntomas psicóticos o depresivos "de novo", crisis convulsivas o estatus epiléptico refractario, o demencia rápidamente progresiva. Las encefalitis autoinmunes están asociadas a diversos fenómenos desencadenantes, como infecciones virales previas entre las más comunes, y se asocian con la presencia de anticuerpos antineuronales y/o onconeuronales, que deben estudiarse ante la sospecha de esta entidad. Es muy importante desarrollar un diagnóstico presuntivo y precoz, ya que la terapia con inmunosupresores como los corticoides -iniciados en el momento oportuno-, puede cambiar su evolución hacia la mejoría clínica. Presentamos un paciente con encefalitis autoinmunes y anticuerpos anti-Titina positivos (habitualmente presentes en timoma y miastenia gravis), no asociados a neoplasia conocida y con buena respuesta a esteroides.

Autoimmune Encephalitis, are an emerging condition, characterized by the sudden onset of psychotic or depressive symptoms "de novo", refractory seizures or epilepsy, or rapidly progressive dementias. The autoimmune encephalitis are associated to various triggered phenomena as a previous viral infections among others; it's related to the presence of antineuronal and/or onconeuronal antibodies, and there must be studied when autoimmune encephalitis is suspected. It is very important to develop a presumptive and early diagnosis, since steroid therapy -on opportunity time- can change its evolution towards clinical improvement. We present a patient with autoimmune encephalitis, and positive anti-Titin antibodies (usually presents in thymoma and myasthenia gravis) not associated with known neoplasia, and with a good response to steroids.

Clinical features of autoimmune encephalitis secondary to epidemic encephalitis B in 5 children / 中国当代儿科杂志

OBJECTIVES@#To study the clinical features of children with autoimmune encephalitis (AE) secondary to epidemic encephalitis B (EEB).@*METHODS@#A retrospective analysis was performed on the medical data of five children with EEB with "bipolar course" who were treated in Children's Hospital Affiliated to Zhengzhou University from January 2020 to June 2022.@*RESULTS@#Among the five children, there were three boys and two girls, with a median age of onset of 7 years (range 3 years 9 months to 12 years) and a median time of 32 (range 25-37) days from the onset of EEB to the appearance of AE symptoms. The main symptoms in the AE stage included dyskinesia (5/5), low-grade fever (4/5), mental and behavioral disorders (4/5), convulsion (2/5), severe disturbance of consciousness (2/5), and limb weakness (1/5). Compared with the results of cranial MRI in the acute phase of EEB, the lesions were enlarged in 3 children and unchanged in 2 children showed on cranial MRI in the AE stage. In the AE stage, four children were positive for anti-N-methyl-D-aspartate receptor antibody (one was also positive for anti-γ-aminobutyric acid type B receptor antibody), and one was negative for all AE antibodies. All five children in the AE stage responded to immunotherapy and were followed up for 3 months, among whom one almost recovered and four still had neurological dysfunction.@*CONCLUSIONS@#EEB can induce AE, with anti-N-methyl-D-aspartate receptor encephalitis as the most common disease. The symptoms in the AE stage are similar to those of classical anti-N-methyl-D-aspartate receptor encephalitis. Immunotherapy is effective for children with AE secondary to EEB, and the prognosis might be related to neurological dysfunction in the acute phase of EEB.

Clinical characteristics of 31 patients with anti-leucine-rich glioma inactivated-1 antibody-associated encephalitis / 临床神经病学杂志

Objective To improve the clinical recognition of anti-leucine-rich glioma inactivated-1(LGI1)antibody associated encephalitis through investigating the clinical characteristics of the disease,so as to improve diagnosis and prognosis.Methods A total of 31 consecutive patients with anti-LGI1 antibody associated encephalitis were recruited during January 2020 to March 2022 in Brain Hospital Affiliated to Nanjing Medical University including their clinical manifestations.Patients were divided into first-line immunotherapy group and mycophenolate mofetil(MMF)adding-on group according to the initial treatment regimen.Univariate and multivariate COX regression analysis were used to analyze the factors of the recurrence of anti-LGI1 antibody encephalitis.mRS was used to evaluate the prognosis.Results Seizure(93.5%),memory defect(80.6%)and psychosis(58.1%)were the most common manifestations in 31 patients.Fourteen patients suffered with hyponatremia,1 patients with elevated white cells and 17 patients with elevated proteins in CSF.The corresponding positive rates of anti-LGI1 antibody were 90.3%and 93.1%in serum and CSF.Fourteen patients had abnormal cranial MR.All patients received first-line immunotherapy,with 13 patients followed by MMF.Eleven patients were recurred in the follow-up.Univariate COX regression analysis found female(HR =3.85,95%CI:1.12-13.24,P =0.032),MMF adding-on therapy(HR = 4.03,95%CI:1.07-15.22,P = 0.04)were associated with relapse.However no independent associations were found after multivariate COX regression analysis.Twenty-two patients had sequeleas during follow-up,with memory decline as the most common symptom.All patients achieved good prognosis.Conclusions Anti-LGI1 antibody associated encephalitis may have good prognosis,but relapse is not rare.The majority patients have sequelaes,with memory defect and psychosis being the most common symptoms.MMF might have no prevention effect on anti-LGI1 antibody encephalitis recurrence.

Plasmaféresis terapéutica en paciente pediátrico con encefalitis autoinmune / Therapeutic apheresis in pediatric patient with autoimmune encephalitis

La encefalitis autoinmune es un cuadro con una expresión neuropsiquiátrica especialmente en pediatría, aunque existen diversas opciones de tratamiento, otras alternativas terapéuticas se relacionan con procedimientos que pueden tener un mayor beneficio para el paciente, como es la plasmaféresis. Este procedimiento, representa una de las terapias de primera línea en este padecimiento. Hace poco se cuenta con la disponibilidad de estos equipos, motivo por el cual describimos este primer procedimiento en pediatría a nivel Bolivia describiendo el manejo en un paciente con encefalitis autoinmune.

Autoimmune encephalitis is a condition with a neuropsychiatric expression, especially in pediatrics, although there are various treatment options, other therapeutic alternatives are related to procedures that may have a greater benefit for the patient, such as plasmapheresis. This procedure represents one of the first line therapies in this condition. This equipment has recently become available, which is why we describe this first procedure in pediatrics at the Bolivian level, describing the management of a patient with autoimmune encephalitis.

Clinical characteristics of autoimmune encephalitis with mental symptoms / 中风与神经疾病杂志

@#To analyze the characteristics of mental symptoms in patients with autoimmune encephalitis with different antibody types. Methods The clinical characteristics of patients with autoimmune encephalitis were retrospectively studied. The mental symptoms of patients with autoimmune brain were analyzed. Subjects：Patients diagnosed with autoimmune encephalitis admitted to the Department of Neurology，the First Affiliated Hospital of China Medical University from September 2019 to December 2020.Results There were 20 cases with positive antibody，and the most common clinical manifestations were convulsion，mental symptoms，consciousness disorder and memory decline. One tumor was found in NMDAR AE，which was teratoma of ovary. Conclusion The mental symptoms of patients with subtypes of autoimmune encephalitis have no significant difference，that is，the type of autoimmune brain antibodies can not be determined by the patients’ major mental symptoms.

Anti-mGluR5 encephalitis with mental disorders as the initial symptom： a case report / 四川精神卫生

This article reported the clinical features of a rare patient with anti-metabotropic glutamate receptor 5 （mGluR5） encephalitis with mental disorders as the initial symptom， so as to provide references for clinical diagnosis and treatment. The patient was a 38-year-old male， developed pharyngeal pain as prodromal symptoms， and the main clinical manifestations included rapidly progressive memory loss， anxiety and depression， and psychomotor excitement symptoms including irritability and impulsive behaviors. The disease had a progressive deterioration. In the most severe state， the patient became unconscious in a shallow coma， with further cognitive decline， hallucinations and delusions， and lack of self-awareness. Both cerebrospinal fluid and serum anti-mGluR5 antibody were strongly positive （1∶100）. After two sessions of hormone shock therapy， the patient showed significantly improvement in consciousness， cognitive， emotional and psychiatric dimensions.

Clinical analysis of 14 patients with anti-metabotropic glutamate receptor 5 encephalitis / 中华神经医学杂志

Objective:To summarize the clinical and imaging characteristics of anti-metabotropic glutamate receptor 5 (mGluR5) encephalitis, and improve clinicians' understanding of this encephalitis.Methods:The clinical data of one patient with anti-mGluR5 encephalitis admitted to our hospital from December 2020 were analyzed, and the data of 13 patients with anti-mGluR5 encephalitis reported in domestic and foreign databases were summarized and analyzed.Results:The main symptoms of 14 patients with anti-mGluR5 encephalitis included mental and behavior disorder (13/14), cognitive dysfunction (11/14), sleep dysfunction (9/14), seizures (8/14), decreased level of consciousness (6/14) and motor disorders (4/14). Four patients had abnormal brain MRI, including hyperintensity in the right mesial temporal lobe on T2 sequences, hyperintensity in the bilateral upper pons on T2 sequences, hyperintensity in the bilateral posterior parietal-occipital cortex on FLAIR sequences, and hyperintensity in the bilateral frontal lobes, right occipital lobe and cerebellum on T2/FLAIR sequences. Six patients had Hodgkin's lymphoma. Treatments included immunotherapy and oncologic therapy. Relapse was noted in 3 of the 14 patients, and symptoms improved significantly after immunotherapy. Except for one patient who died in hospital, 6 patients showed complete recovery and 7 patients showed partial recovery at the last follow-up (median 20 months).Conclusions:Anti-mGluR5 encephalitis is rare autoimmune encephalitis whose symptoms are not limited to the limbic system, and relapse may occur; immunotherapy has good effects; Hodgkin's lymphoma is often associated in these patients.

Clinical characteristics of autoimmune glial fibrillary acidic protein astrocytopathy / 中华神经医学杂志

Objective:To analyze the clinical features of autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A).Methods:A retrospective analysis was performed. Data of 14 patients with GFAP-A, admitted to Department of Neurology, Chenzhou First People's Hospital from December 1, 2020 to May 1, 2022, were collected.Results:The mean age of these 14 patients was (46±31) years, and the male patients were more than female ones (10∶4). All patients had acute onset, and the main clinical symptoms included dizziness and headache ( n=10), fever ( n=8), urination and defecation function disturbance ( n=5), mental and behavioral abnormality ( n=4), limb weakness ( n=4), and tremor ( n=3). No tumors were found; 8 patients were with hyponatremia. Twelve patients responded well to hormone and/or immunosuppressive therapy; 2 patients refused corticosteroid and/or immunosuppressive therapy for personal reasons, and their condition did not improve at discharge. Conclusion:In these GFAP-A patients, relatively heterogeneous clinical manifestations are noted; many are complicated with hyponatremia, and some have clinical manifestations similar to nervous system infectious diseases; the prognosis is good after immunotherapy.