Form Of Autoimmune Encephalitis Research Articles

A complex and severe encephalitis associated with four co-existing neuronal cell-surface autoantibodies

Many forms of autoimmune encephalitis are mediated by neuronal cell-surface directed autoantibodies. The co-occurrence of four neuronal cell-surface antibodies in a single patient is exceptionally rare. We report a patient who had a severe encephalitis associated with antibodies to NMDA, Glycine, GABAA and GABAB receptors. Case: A 28-year-old man on tacrolimus presented with a first seizure. Thereafter, he developed confusion, cerebellar signs, opsoclonus, neuromyotonia and medication-refractory seizures. CSF sampling revealed 826 white cells and NMDA, glycine and GABAB receptor antibodies: all were also detected in serum along with additional GABAA receptor antibodies. Neural antibodies were detected using fixed (NMDA, GABAA, GABAB receptor) or live (glycine receptor) cell-based assays at Oxford Neuroimmunology Laboratory, Oxford, UK. MRI brain demonstrated cerebellar leptomeningeal enhancement and a hyperintense lesion in the cerebellar vermis. EEG revealed extreme delta brush and needle EMG confirmed neuromyotonia. No underlying malignancy was detected. Methylprednisolone, IVIG, Rituximab, therapeutic plasma exchange, cyclophosphamide and bortezomib were administered sequentially, with minimal clinical improvement. Death secondary to respiratory sepsis occurred on the 714th hospital day. Postmortem revealed pan-cerebellar atrophy with Purkinje cell loss; dentate nucleus ganglionopathy, and thoracolumbar cord myelopathy. In summary, the detection of multiple neuronal cell-surface antibodies in autoimmune encephalitis is unusual and may result in a complex overlap syndrome with a poor response to immunotherapy.

Anti-NMDA receptor encephalitis as a reversible cause of acute psychoses: Diagnostic possibilities. (Comment on the article by E.V. Snedkov "Is anti-NMDA receptor encephalitis real? I. Diagnostic challenges")

Encephalitis with antibodies to NMDA receptors is a form of autoimmune encephalitis that most often debuts as an acute psychosis. Considering its curability and reversibility, encephalitis with antibodies to NMDA receptors must be included into differential diagnostic list for all the patients suffering first psychotic episode. However, to diagnose it, it is necessary to follow a step-by-step algorithm considering both clinical and instrumental and laboratory data. Confirming anti-NMDA-receptor encephalitis based solely on increased blood antibodies level often leads to false-positive diagnostics and errors in treatment of such patients. In this article, we discuss diagnostic features of encephalitis with antibodies to NMDA receptors,as well as other forms of autoimmune encephalitis that can initially manifest as acute psychoses.

A case report of anti-NMDA receptor encephalitis in a young Egyptian female patient presenting with hyperreligiosity

Anti-n-methyl-d-aspartate receptor (NMDAR) encephalitis is a form of autoimmune encephalitis that remains under-recognized due to the variability of the initial symptoms and can be misdiagnosed as viral encephalitis or other pathogens. This syndrome has been predominantly described in young females including personality changes, autonomic dysfunctions, and neurologic decompensation.About half of the cases have tumors, most commonly teratomas of the ovaries; another established trigger is herpes viral encephalitis, while the cause in other cases is unclear. In case of clinical suspicion, electroencephalogram and brain magnetic resonance imaging are useful, but lumbar puncture for cerebrospinal fluid analysis is used to confirm the diagnosis. Treatment for this disease includes immunosuppression, plasmapheresis, and tumor resection when indicated. In this case report, we present a case that presented with hyperreligiosity and proved to have autoimmune encephalitis.The main purpose of our case is to increase awareness regarding immune-mediated encephalitis, especially the anti-NMDAR encephalitis.

Experience with anti NMDA receptor antibody encephalitis in a tertiary care hospital in Pakistan

Background and objective: 
 Anti NMDA receptor (Anti-NMDAR) antibody encephalitis is an increasingly recognized form of auto immune encephalitis. The objective of this study was to determine the demographic, clinical and laboratory factors associated with this syndrome. 
 Methods:
 All cases presenting to neurology, Pakistan institute of medical sciences, Islamabad, from June 2017 till January 2020 were reviewed retrospectively. Patients fulfilling the diagnostic criteria for Anti NMDA receptor (Anti-NMDAR) antibody encephalitis were included. All patients were evaluated with cerebrospinal fluid (CSF)routine examination, MRI brain, autoimmune encephalitis profile and for presence of oligoclonal bands (OCB) in CSF. Statistical analysis was done using SPSS version 23.0.
 Results:
 A total of 7 patients were diagnosed as having Anti-NMDARantibody encephalitis. Five patients were female and two were male. Four patients had some type of psychiatric disturbances upon presentation of these behavioral abnormalities and irritability were found in all four. Orofacial abnormal movements were found in four patients while one patient had myoclonic seizures.
 Conclusion:
 Anti-NMDARreceptor encephalitis is a common cause of encephalitis associated with neurological and psychiatric symptoms. Delay in diagnosis can occur due to non-specific symptoms and signs present in early stages of illness,this can lead to long term neurological disability.

Brain blood vessel autoantibodies in patients with NMDA and GABAA receptor encephalitis: identification of unconventional Myosin-X as target antigen.

Introduction: The antibody repertoire from CSF-derived antibody-secreting cells and memory B-cells in patients with encephalitis contains a considerable number of antibodies that do not target the disease-defining autoantigen such as the GABA or NMDA receptors. This study focuses on the functional relevance of autoantibodies to brain blood vessels in patients with GABAA and NMDA receptor encephalitis. Methods: We tested 149 human monoclonal IgG antibodies from the cerebrospinal fluid of six patients with different forms of autoimmune encephalitis on murine brain sections for reactivity to blood vessels using immunohistochemistry. Positive candidates were tested for reactivity with purified brain blood vessels, effects on transendothelial electrical resistance (TEER), and expression of tight junction proteins as well as gene regulation using human brain microvascular endothelial hCMEC/D3 cells as in vitro blood-brain barrier model. One blood-vessel reactive antibody was infused intrathecally by pump injection in mice to study in vivo binding and effects on tight junction proteins such as Occludin. Target protein identification was addressed using transfected HEK293 cells. Results: Six antibodies reacted with brain blood vessels, three were from the same patient with GABAAR encephalitis, and the other three were from different patients with NMDAR encephalitis. One antibody from an NMDAR encephalitis patient, mAb 011-138, also reacted with cerebellar Purkinje cells. In this case, treatment of hCMEC/D3 cells resulted in decreased TEER, reduced Occludin expression, and mRNA levels. Functional relevance in vivo was confirmed as Occludin downregulation was observed in mAb 011-138-infused animals. Unconventional Myosin-X was identified as a novel autoimmune target for this antibody. Discussion: We conclude that autoantibodies to blood vessels occur in autoimmune encephalitis patients and might contribute to a disruption of the blood-brain barrier thereby suggesting a potential pathophysiological relevance of these antibodies.

Case report of anti-NMDA receptor encephalitis in a 24-year-old female: an uncommon presentation

BackgroundAnti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a form of autoimmune encephalitis. Due to the variability of the initial symptoms, anti-NMDAR encephalitis is not only underdiagnosed but also can be misdiagnosed as viral encephalitis or other pathologies. The origin of this disease is often paraneoplastic. Anti-NMDAR encephalitis preferentially affects children and young adults, and it has a male/female ratio of 1/4. In case of clinical suspicion, electroencephalogram and brain magnetic resonance imaging are useful, but lumbar puncture for cerebrospinal fluid analysis is used to confirm the diagnosis. Treatment for this disease includes immunosuppression and tumour resection when indicated.Case presentationWe report the case of a 24-year-old female admitted to the emergency room following the onset of acute confusion. Due to the rapid deterioration of consciousness, swallowing disorders, respiratory failure and severe bradycardia the patient was intubated. On day 23 after presentation, brain magnetic resonance suggested autoimmune limbic encephalitis. Cerebrospinal fluid results were positive for anti-NMDA antibodies. After IV methylprednisolone and plasmapheresis and a second line therapy with corticosteroid and mycophenolic acid, the patient’s clinical condition gradually improved.ConclusionsAnti-NMDAR encephalitis typically occurs in young patients with no history of acute psychiatric symptoms. The possibility of this pathology should be taken into account before diagnosing a patient with a psychiatric illness.

P.026 Autoimmune encephalitis: modifiable and non-modifiable predictors of relapse

Background: Approximately 25% of encephalitis cases in North America are immune mediated. For most forms of autoimmune encephalitis (AIE), risk of relapse is unclear and little evidence exists to guide which patients have the highest risk and whether standard treatments reduce this risk. Our objective was to determine the factors associated with AIE relapse. Methods: We performed a chart review consisting of patients with AIE presenting to the Calgary Neuro-Immunology Clinic and Tom Baker Cancer Centre between 2015 and 2020. Predictors of relapse were determined with use of t-test. Results: Outcome data was assessable in 39/40 patients, 17/39 (44%) patients relapsed. Seropositive patients and those with abnormal CSF were more likely to relapse, although neither reached statistical significance (p=0.12, 0.059). Patients with longer duration of steroid and steroid sparing treatment prior to relapse, and those on steroids at the time of relapse, had milder relapses (p=0.024, 0.026, 0.047). There was no difference in steroid or steroid sparing treatment use at 3, 6, and 12 months between groups. Conclusions: Risk of relapse in AIE is high (44%), with most relapses occurring in the first 3 years. Continuous immunosuppression lessens the severity of relapse, although our study did not confirm it reduced the occurrence of relapse.

Autoimmune Encephalitis.

Autoimmune encephalitis is a common and treatable cause of encephalitis in children and adults. Individuals present with a variety of symptoms, including altered mental status, behavioral changes, irritability, insomnia, developmental regression, seizures, dyskinetic movements, and autonomic instability. Evaluation includes electroencephalography, magnetic resonance imaging, and lumbar puncture. Once infectious and other causes are reasonably ruled out, treatment should be started empirically without waiting for antibody confirmation. Early clinical suspicion is key, as the outcome depends on early initiation of immunotherapy, including corticosteroids, intravenous immunoglobulin, and/or plasmapheresis. Severe or refractory cases require other treatments, such as rituximab, cyclophosphamide, or other immunotherapies using novel monoclonal antibodies. Psychiatry should be involved early for the management of behavioral issues. Additional considerations include management of seizures and dyskinesias. ICU admission may be required for management of hypoventilation necessitating mechanical ventilation (either intrinsic or iatrogenic, eg, from sedatives), refractory seizures, and dysautonomia. Anti-N-methyl-d-aspartate receptor and other forms of autoimmune encephalitis are less often associated with neoplasia (such as ovarian teratoma) in children compared with adults, but screening and removal of tumor if present should be performed.

Anti-NMDA Receptor Encephalitis: Retrospective Analysis of 15 Cases, Literature Review, and Implications for Gynecologists.

Background Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a rare form of autoimmune encephalitis caused by anti-NMDA receptor antibodies. This disease mainly affects women of childbearing age and is commonly associated with ovarian teratoma. However, the relationship between anti-NMDA receptor encephalitis and ovarian teratoma and the role of anti-NMDA receptor antibody in the relationship remain unclear. Objectives This study aimed to describe 15 cases of anti-NMDA receptor encephalitis (5 with ovarian teratoma), review literature, and reinforce the gynecologist's knowledge of this disorder. Methods Clinical data of 15 patients from January 2015 to December 2020 admitted to The Second Hospital of Hebei Medical University were collected and analyzed. The diagnosis of anti-NMDA receptor encephalitis was based on the presence of anti-NMDA receptor antibodies in cerebrospinal fluid (CSF) and/or serum. Laparoscopic teratoma removal was performed in patients with ovarian teratoma. All patients had received immunotherapy. In addition, a review of the literature was performed to reinforce the gynecologist's knowledge of this disorder. Results A total of 15 patients with anti-NMDA receptor encephalitis were screened, of whom 5 patients were confirmed with ovarian teratoma by pathology. The most common symptoms of anti-NMDAR encephalitis with teratoma are fever (5/5, 100%), seizure (5/5, 100%), mental and behavioral disorders (4/5, 80%), and decreased consciousness (4/5, 80%). Conversely, the most common symptoms of patients without teratoma were neuropsychiatric symptoms, including headache (6/10, 60%) and mental and behavioral disorders (7/10, 70%). All patients underwent immunotherapy, including steroids, intravenous immunoglobulin (IVIG), plasma exchange, and cyclophosphamide, and 4 out of 5 patients with ovarian teratomas underwent surgical treatment. All patients had a good outcome after systemic, surgical, and immunotherapy treatment. No patient who underwent surgical treatment developed a recurrence. Conversely, 2 of 10 patients without teratoma developed an anti-NMDA receptor encephalitis recurrence. Conclusions Patients with anti-NMDA encephalitis show severe mental and neurological symptoms. Resection of teratoma is beneficial to the relief or disappearance of symptoms and has a good prognosis. This disorder should be fully recognized by gynecologists, who play an important role in diagnosis and treatment.

Putative autoimmune mechanisms for Acute Disseminated Encephalomyelitis (ADEM) and Guillain-Barré Syndrome (GBS) associated with SARS-CoV-2 infection

The novel coronavirus severe acute respiratory syndrome coronavirus 2, SARS-CoV-2, responsible for the ongoing COVID-19 pandemic, is associated with a broad manifestation of neurological disease, including Acute Disseminated Encephalomyelitis (ADEM) and Guillain-Barré Syndrome (GBS), amongst other forms of autoimmune encephalitis, stroke, encephalopathy, delirium, and cranial neuropathies. These phenomena are not limited to human coronaviruses but are also seen in a minority of patients in response to other viral infection. There is good evidence that an autoimmune mechanism hypothesis is likely. The final pathology is probably the culmination of mixed mechanisms such as vascular and immune dysregulation as well as direct viral invasion of neurons – though there is little if any evidence of viral invasion in the literature to date. The aim of this review is to elucidate the emerging evidence about this subset of COVID-19-associated neurological disease. This unique opportunity to study the interactions between virus and host immune and central nervous system (CNS) to gain novel insights applicable to other probable autoimmune neurological disease. I have conducted a literature search as well as drawn on my own observations from the COVID-19 and encephalitis multidisciplinary meetings at Queen Square National Hospital for Neurology and Neurosurgery, London, UK.

Проблемы диагностики аутоиммунной эпилепсии

Objective of the Review: To analyse and systematise knowledge in the problems with autoimmune epilepsy diagnostics. Main part. Clinical phenotypes of immune-mediated epilepsy depend on various types of antibodies. However, autoimmune epilepsy is diagnosed also in patients who are followed up for chronic refractory unexplained epilepsy, especially in initial epileptic status and late onset epilepsy without structural changes on brain imaging. Brain imaging changes may not be observed, especially in early disease. Very often a diagnostic challenge in autoimmune epilepsy is the difference between epileptic seizures and behavioural symptoms and the mental changes caused by involvement of limbic brain structures. An important role in detection of seizures and differential diagnosis is played by video-EEG-monitoring, which allows identifying the true number of seizures, epileptiform activity between seizures, behavioural changes not related to paroxysmal activity of cortical neurons. Any specific EEG signs for differentiation between various types of autoimmune epilepsy have not been found yet. Still, EEG can provide patterns that are unique for certain forms of autoimmune encephalitis. Conclusion. Video-EEG-monitoring significantly contributes to autoimmune epilepsy diagnostics, and some changes can be used as markers of disease severity. It is very important, especially in patients with impairment of consciousness, where identification of the clinical status and response to therapy is challenging. Keywords: autoimmune epilepsy, video-EEG-monitoring, epilepsy diagnostics

Anti-NMDA Receptor Encephalitis Associated With an Ovarian Teratoma Presenting as First-episode Psychosis: A Case Report

We report an unusual case of a 27-year-old previously healthy female who presented with a 15-day history of psychotic, cognitive, and unspecified somatic symptoms. She was admitted to the psychiatric ward of an early intervention in psychosis team and medicated with aripiprazole. The young age of onset, the rapid onset, the absent history of psychiatric disease, and the persistence of a marked memory deficit after the psychotic symptoms remitted strongly suggested a nonpsychiatric etiology and led us to hypothesize autoimmune encephalitis as the most probable diagnosis. An investigation was carried out for anti-N-methyl-D-aspartate (anti-NMDA) receptor antibodies in the patient's serum and cerebrospinal fluid, and both tests were positive. The patient was transferred to the neurology ward, where an endovaginal ultrasound showed an ovarian teratoma in her right ovary. She underwent laparoscopic surgery without complications. She was initially treated with intravenous immunoglobulin and methylprednisolone for 5 days, which resulted in marked improvement of her memory and attention performance. Anti-NMDA receptor encephalitis, first described in 2007 by Dalmau and colleagues, is a form of auto-immune encephalitis with prominent neuropsychiatric manifestations, particularly psychotic symptoms. At symptom onset, distinguishing the disease from a primary psychiatric disorder is challenging. This case report highlights the importance of early psychosis treatment teams considering the diagnosis of anti-NMDA receptor encephalitis when evaluating new referrals with a potential diagnosis of first-episode psychosis, particularly when young patients with no relevant personal or familial psychiatric history present with neuropsychiatric symptoms and a distinctive pattern of symptom fluctuation over time.

The Spectrum of Movement Disorders in Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis Both in Children and Adults: An Experience From a Single Tertiary Care Center.

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a form of autoimmune encephalitis. The characteristic clinical features include seizure, psychosis-like symptoms, abnormal movements, and autonomic disturbances. Patients with anti-NMDAR encephalitis can present with various types of movement disorders. Typically, the movement disorders start following intervals of psychiatric and prodromal manifestations in young adults; however, in children, these might be an early presentation of anti-NMDAR encephalitis. The disease is under-recognized and underdiagnosed in Pakistan. Early recognition of the disease is important to commence timely treatment leading to a better prognosis. Here we present a collection of anti-NMDAR encephalitis patients, specifically focussing on the different types of movement disorders and the differences in clinical manifestations between children and adults.

Practice-relevant autoimmune diseases of the central nervous system in pediatrics: early diagnosis and adequate initiation of treatment

Pediatric autoimmune diseases affecting the central nervous system have recently come into the the focus of attention. Important advances have been made in the field of children with multiple sclerosis (MS), which led to abetter understanding of the clinical characteristics and treatment options. Furthermore, new autoantibodies against target antigens of neurons, peripheral nerves and the myelin sheath have been detected. This article summarizes new advances in children with MS and addresses the differences to their adult counterparts. In addition, the most important forms of autoimmune encephalitis, such as N‑methyl D‑aspartate receptor (NMDA-R) or myelin oligodendrocyte glycoprotein (MOG) encephalitis in children are described together with the diagnostic algorithm and therapeutic approach in the event of asuspected autoimmune encephalitis. Lastly, the clinical spectrum of MOG antibody-associated diseases (MOGAD) is detailed.

P.004 Autoimmune Encephalitis: Modifiable and Non-Modifiable Predictors of Relapse

Background: Approximately 25% of encephalitis cases in North America are immune mediated. For most forms of autoimmune encephalitis (AIE), risk of relapse is unclear and little evidence exists to guide which patients have the highest risk and whether standard treatments reduce this risk. Our objective was to determine the factors associated with AIE relapse. Methods: We performed a chart review consisting of patients with AIE presenting to the Calgary Neuro-Immunology Clinic and Tom Baker Cancer Centre between 2015 and 2020. Predictors of relapse were determined with use of t-test. Results: Outcome data was assessable in 39/40 patients, 17/39 (44%) patients relapsed. Seropositive patients and those with abnormal CSF were more likely to relapse, although neither reached statistical significance (p=0.12, 0.059). Patients with longer duration of steroid and steroid sparing treatment prior to relapse, and those on steroids at the time of relapse, had milder relapses (p=0.024, 0.026, 0.047). There was no difference in steroid or steroid sparing treatment use at 3, 6, and 12 months between groups. Conclusions: Risk of relapse in AIE is high (44%), with most relapses occurring in the first 3 years. Continuous immunosuppression lessens the severity of relapse, although our study did not confirm it reduced the occurrence of relapse.

Autoimmune Encephalitis and Acute Rituximab Treatment

Few randomized, controlled trials have been conducted to test treatments for the various forms of autoimmune encephalitis (AE). Investigators have now

Anti-NMDAR Encephalitis with Relapsing Optic Neuritis

Autoimmune encephalitis with antibodies to NMDA receptors (anti-NMDAR encephalitis) is a frequent form of autoimmune encephalitis. The disease is curable, though the lack of timely treatment can lead to disability in patients or death. Difficulties in the diagnosis of anti-NMDAR encephalitis arise from its heterogenous manifestations, its possible combination with other autoimmune diseases, and lack of doctors’ awareness of this type of encephalitis. This article reviews published data and describes a case of anti-NMDAR encephalitis with atypical features in the form of relapsing optic neuritis; it was difficult to link optic nerve lesions with comorbidity with multiple sclerosis or optic neuromyelitis spectrum disorders.

Recognizing psychiatric presentations of anti-NMDA receptor encephalitis in children and adolescents: A synthesis of published reports.

The aim of this study was to improve early recognition of anti-N-methyl-d-aspartate receptor encephalitis (anti-NMDArE) in children and adolescents by identifying characteristic temporal patterns of clinical features in patients likely to be referred for psychiatric evaluation. In this form of autoimmune encephalitis, NMDAr hypofunction is caused by autoantibodies to receptor surface components. Clinical outcomes following prompt immunotherapy are usually good, but delayed treatment often results in a protracted course with significant residual disability or death. Anti-NMDArE frequently mimics psychiatric disorders, so most patients are referred initially to a psychiatrist and treated for days or weeks before being correctly diagnosed. A systematic search of PubMed and EMBASE electronic databases identified all published reports of antibody-confirmed anti-NMDArE associated with psychiatric symptoms in patients <19 years old. Redundant reports were eliminated manually. For each patient, the order in which each feature was first observed was ranked relative to others. Median temporal ranks were used to compare the sequencing of individual features and major symptom domains. One hundred and sixty seven cases (121 females) met the inclusion criteria. The most common features were dyskinesias (77.8%), seizures (72.5%), mutism or staring (40.7%), insomnia (39.5%), language dysfunction (36.5%), fever (31.1%), disorientation/confusion (28.7%), reduced arousal (28.1%), and memory disturbance (26.9%). The configuration and temporal sequencing of features were highly variable between individuals. Clinicians need to suspect this disorder: if new behavioral symptoms arise in the context of a recent viral prodrome; if they are accompanied by dyskinesias, seizures or insomnia; or if psychiatric symptoms are unusual (e.g., non-verbal auditory hallucinations).

Anti-NMDA receptor encephalitis presenting as an acute psychotic episode misdiagnosed as dissociative disorder: a case report.

BackgroundIn 2005, “anti-N-methyl-d-aspartate (NMDA) receptor encephalitis,” a syndrome with prominent psychiatric symptoms, memory loss, decrease in level of consciousness, and central hypoventilation, was described in young women with ovarian teratomas and antibodies against an antigen highly expressed in the hippocampus. This report highlights the growing need for increased awareness among psychiatrists and other relevant medical professionals about this under-diagnosed disorder, which should be considered in differential diagnoses.Case presentationA 19-year-old female with no psychiatric history presented to a district general hospital with acute psychosis, emotional lability, memory deficit, fluctuating behavioral changes such as wandering and babbling, and seizure. She was admitted to the hospital with a provisional diagnosis of dissociative disorder. Soon after admission, she developed aspiration pneumonia and was intubated for mechanical ventilation. She was transferred to our hospital for further assessment and admitted to the intensive care unit for ventilation. Laboratory test results were unremarkable, but her EEG showed non-specific slowing with no epileptiform activity, and brain computed tomography (CT) and MRI also showed no remarkable findings. Cerebrospinal fluid (CSF) analysis showed an elevated white blood cell count (15 cells/hpf; 70 % lymphocytes), and blood serum and CSF samples tested positive for NMDA receptor antibodies. Abdominal contrast-enhanced CT revealed an ovarian teratoma, which was subsequently removed laparoscopically. Postoperative immunotherapy (steroids, intravenous immunoglobulin, and plasmapheresis) led to gradual improvement. On day 25 of hospitalization, neuropsychological assessment demonstrated that overall, she had returned to her premorbid level of functioning. Her condition substantially improved over several months of cognitive rehabilitation, and she was eventually discharged on day 75.ConclusionsAnti-NMDA receptor encephalitis, a form of autoimmune encephalitis, is commonly associated with tumors and often misdiagnosed. Diagnosis can be confirmed by detecting NMDA receptor antibodies in the patient’s serum or CSF. Management can be achieved with immunosuppressive therapy and tumor resection.

Pathophysiology and Prognostic Factors of Autoimmune Encephalitis

More and more forms of autoimmune encephalitis are being identified with the clinical spectrum ranging from epilepsy over movement disorders to psychosis. The increasing appreciation of clinical symptoms raises questions about the underlying pathophysiological mechanisms and prognostic factors. Numerous novel findings on the aetiology demonstrate that diverse tumours, but also infections of the central nervous system such as Herpes encephalitis can trigger autoimmune encephalitis. Antibodies against neuronal surface epitopes are directly pathogenic in the majority of cases. They act via binding and internalization of target proteins, receptor blockage, or activation of complement. Most relevant for the patients' prognosis are the type and titer of antibodies (e. g. against NMDA, GABA, AMPA receptors or voltage-gated potassium channel complexes), associated tumours, sufficiently aggressive immunotherapies, and imaging as well as cerebrospinal fluid biomarkers.