Treatment Of Autoimmune Encephalitis Research Articles

Efficacy of ofatumumab combined with corticosteroids in the treatment of autoimmune encephalitis: a 6-month cohort study.

This study investigated the efficacy of ofatumumab (OFA) combined with corticosteroids in autoimmune encephalitis (AE) patients refractory to conventional treatment. Eighteen AE patients admitted to Ruijin Hospital between June 2022 and September 2023 received four subcutaneous 20-mg OFA injections at 0, 1, 6, and 12 weeks combined with standard corticosteroid therapy. Clinical symptoms, modified Rankin scale (mRS) scores, Clinical Assessment Scale for Autoimmune Encephalitis (CASE) scores, serum immunoglobulin (Ig) levels (IgG and IgM), and peripheral blood CD20 + B cell levels were documented before OFA administration and at 1, 2, 6, 12, and 24 weeks post-treatment. OFA treatment significantly improved psychiatric symptoms (P = 0.025), cognitive dysfunction (P = 0.008), and seizure frequency (P = 0.014). The mRS and CASE scores improved after two injections in patients with anti-NMDAR encephalitis (P = 0.041), but not in patients with anti-LGI1 encephalitis (P > 0.05). The mRS scores significantly improved at 12 weeks post-treatment in antibody-negative encephalitis. Blood CD20 + B cell levels dropped to zero after an average of 2.5 injections. No significant changes could be observed in serum IgG and IgM levels (P > 0.05). Seven patients had mild fever or pulmonary infections post-treatment. This study suggests that OFA is a safe and effective treatment for a subset of AE patients, particularly in cases of anti-NMDAR encephalitis, though further research is needed on long-term outcomes and recurrence.

Diagnostic evaluation of autoimmune encephalitis

Autoimmune encephalitis (AIE) is a complex neurological condition characterized by brain inflammation caused by autoantibodies targeting neuronal surface antigens or synaptic receptors resulting in direct antibody-mediated neuronal dysfunction. The aetiology of AIE includes malignancies and viral infections. However, significant proportion of these patients have no identifiable trigger.The clinical presentation of AIE is varied, ranging from memory deficits and psychiatric symptoms to seizures and movement disorders. The diagnosis is typically based on a combination of supportive clinical features and diagnostic tools such as EEG, MRI, and CSF findings. The detection of antibodies confirms diagnosis of specific autoimmune encephalitis syndromes.Early recognition and prompt treatment of AIE are crucial, as delayed intervention can result in permanent neurological damage. The primary treatment approach is immunotherapy, along with screening for underlying malignancies. With the evolving knowledge on different antibodies and their associated syndromes, it is important for clinicians to have a high degree of suspicion and rule out other differentials to ensure timely and appropriate diagnosis. Furthermore, the use of appropriate diagnostic criteria can help minimize delays in starting treatment.This review provides an overview of the current understanding of AIE, with a focus on pathophysiology, clinical presentation, diagnostics, and treatment modalities, specifically for the most commonly encountered types of AIE associated with cell surface and synaptic antibodies.

Pediatric Autoimmune Encephalitis: A Nationwide Study in Latvia.

Autoimmune encephalitis (AE) is the third most common encephalitis in children. Diagnosis can be challenging due to overlapping and diverse clinical presentations as well as various investigation results. This study aims to characterize the clinical, diagnostic features, as well as treatment and outcomes of AE in children and determine the incidence of pediatric AE in Latvia. The study was conducted at the Children's Clinical University Hospital in Riga. The study participants were patients under the age of 18 years diagnosed with AE from 2014 to 2022. Data regarding clinical characteristics, investigation findings, treatment strategy, and outcomes were retrospectively collected from the medical history data system. We included 18 pediatric patients diagnosed with AE. The mean incidence of pediatric AE in Latvia was 0.56 per 100,000 children. Most patients (66.6%) had seronegative AE. In the seropositive group, the most common was anti-methyl-D-aspartate receptor AE, with two patients having other antibodies. The most prevalent clinical features were personality change, cognitive impairment, autonomic dysfunction, and movement disorders. The majority of patients (58.8%) received first-line treatment only. More than half (55.6%) of our AE patient group had long-term sequelae. Our study shows that the pediatric AE incidence in Latvia is similar to what has been previously reported in other studies. A relatively high proportion of seronegative AE was present in our cohort, indicating that awareness of possible misdiagnosis should be raised. Further research is needed to better understand the underlying mechanisms, characterize clinical features, and determine the treatment of choice in different situations to improve long-term outcomes.

Brazilian consensus recommendations on the diagnosis and treatment of autoimmune encephalitis in the adult and pediatric populations.

Autoimmune encephalitis (AIE) is a group of inflammatory diseases characterized by the presence of antibodies against neuronal and glial antigens, leading to subacute psychiatric symptoms, memory complaints, and movement disorders. The patients are predominantly young, and delays in treatment are associated with worse prognosis. With the support of the Brazilian Academy of Neurology (Academia Brasileira de Neurologia, ABN) and the Brazilian Society of Child Neurology (Sociedade Brasileira de Neurologia Infantil, SBNI), a consensus on the diagnosis and treatment of AIE in Brazil was developed using the Delphi method. A total of 25 panelists, including adult and child neurologists, participated in the study. The panelists agreed that patients fulfilling criteria for possible AIE should be screened for antineuronal antibodies in the serum and cerebrospinal fluid (CSF) using the tissue-based assay (TBA) and cell-based assay (CBA) techniques. Children should also be screened for anti-myelin oligodendrocyte glucoprotein antibodies (anti-MOG). Treatment should be started within the first 4 weeks of symptoms. The first-line option is methylprednisolone plus intravenous immunoglobulin (IVIG) or plasmapheresis, the second-line includes rituximab and/or cyclophosphamide, while third-line treatment options are bortezomib and tocilizumab. Most seizures in AIE are symptomatic, and antiseizure medications may be weaned after the acute stage. In anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, the panelists have agreed that oral immunosuppressant agents should not be used. Patients should be evaluated at the acute and postacute stages using functional and cognitive scales, such as the Mini-Mental State Examination (MMSE), the Montreal Cognitive Assessment (MoCA), the Modified Rankin Scale (mRS), and the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The present study provides tangible evidence for the effective management of AIE patients within the Brazilian healthcare system.

Canadian Consensus Guidelines for the Diagnosis and Treatment of Autoimmune Encephalitis in Adults.

Autoimmune encephalitis is increasingly recognized as a neurologic cause of acute mental status changes with similar prevalence to infectious encephalitis. Despite rising awareness, approaches to diagnosis remain inconsistent and evidence for optimal treatment is limited. The following Canadian guidelines represent a consensus and evidence (where available) based approach to both the diagnosis and treatment of adult patients with autoimmune encephalitis. The guidelines were developed using a modified RAND process and included input from specialists in autoimmune neurology, neuropsychiatry and infectious diseases. These guidelines are targeted at front line clinicians and were created to provide a pragmatic and practical approach to managing such patients in the acute setting.

Navigating the diagnostic and therapeutic complexities of autoimmune encephalitis

Characterized by immune-mediated inflammation of the brain, autoimmune encephalitis (AE) encompasses a diverse array of disorders, each presenting with unique as well as overlapping clinical manifestations, and underlying autoantibodies targeting neuronal and glial surface antigens. The clinical spectrum can range from subtle cognitive and behavioural changes to severe seizures, movement disorders, and altered consciousness. However, what truly sets AE apart from other encephalitides is its potential reversibilitywhen identified early and managed appropriately. The identification of N-methyl-D-aspartate receptor (NMDAR) antibody encephalitis in 2007 heralded the discovery of a spectrum of immunotherapy-responsive AE. However, misdiagnosis remains prevalent despite diagnostic algorithms, resulting in unnecessary immunotherapies and delayed treatment of the correct diagnosis. AE diagnosis requires a judicious blend of diagnostic algorithms, antibody testing, and clinical acumen. Testing for antibodies against neuronal surface antigens, preferably in cerebrospinal fluid, is essential, but should be contextual to clinical relevance.Treatment guidelines are largely based on retrospective studies due to a dearth of randomized and prospective data. However, early immunotherapy initiation has proven to be pivotal for optimal outcomes. The therapeutic landscape in AE continues to evolve with the introduction of novel targeted immunotherapies.This review highlights the complexities, and proposes a systematic approach, to the diagnosis and treatment of AE.

Comparison of intravenous immunoglobulin and plasma exchange in the treatment of autoimmune encephalitis in a tertiary care hospital

Comparison of intravenous immunoglobulin and plasma exchange in the treatment of autoimmune encephalitis in a tertiary care hospital

Thinking in diagnosis and treatment of autoimmune encephalitis in children

Thinking in diagnosis and treatment of autoimmune encephalitis in children

Epileptic manifestations of autoimmune encephalitis

The development of autoimmune encephalitis (AIE) is due to the formation of intracellular and extracellular neuronal antibodies to various structures of the brain tissue. Their prevalence and morbidity are comparable to infectious ones, and the detection rate has recently been increasing. Acute symptomatic seizures are an important component of the clinical core of AIE and are associated with a heterogeneous group of autoantibodies, which along with the features of the lesion topic causes a signi fi cant clinical variety of seizures. The EEG has ictal and interictal features, and the development of electrographic subclinical seizures is also possible. The basis of the treatment of AIE with epileptic seizures is immunotherapy along with the use of antiepileptic drugs with sodium channel blocking properties.

Main Syndrome and Antibody Effects of Autoimmune Encephalitis Caused by Antineuronal Surface Antibodies: An Update

Since the discovery of a series of antineuronal surface antibodies (NSAs), the diagnostic approach and management of patients with autoimmune encephalitis (AE) and related disorders have undergone a "paradigm shift." However, recent topics described below are also announcing the dawn of the next era in the practice of patients with AE. As the clinical spectrum of NSA-associated AE expands, some types of AE (e.g., anti-DPPX antibody-associated and anti-IgLON5 antibody-associated disorders) can be misclassified into reconsider diagnosis when using the previously published diagnostic criteria. Nobel active immunization animal models of NSA-associated disorders (e.g., anti-NMDAR encephalitis model) can remarkably emphasize the understanding of the pathophysiological effects and main syndrome induced by NSAs. Additionally, several international clinical trials (e.g., rituximab, inebilizumab, ocrelizumab, bortezomib, and rozanolixizumab) for AE treatments, including anti-NMDAR encephalitis, have been implemented. Data from these clinical trials can be used to establish the best treatment of AE.

Therapeutic Plasma Exchange in Treatment of Autoimmune Encephalitis: A Case Report

The present study highlights the Therapeutic Plasma Exchange in treatment of Autoimmune Encephalitis. Due to the similarities in the clinical, imaging, and laboratory findings of many forms of autoimmune and infectious encephalitis, autoimmune encephalitis is frequently a difficult clinical diagnosis. Diagnosis of autoimmune encephalitis is based on the clinical course, serological evidence of autoimmunity, EEG changes, evidence of intrathecal inflammation in the cerebrospinal fluid and neuroimaging by MRI. In view of the suspected diagnosis of Autoimmune Encephalopathy with super refractory Status Epilepticus, Methylprednisolone was later added to the treatment. This case represents an interesting scenario of sero-negative encephalitis in which an early diagnosis could be made and treatment initiated in time.

Associations of Serum Neurofilament Light Chain with Outcomes in Children with Autoimmune Encephalitis (P4-5.009)

<h3>Objective:</h3> To examine associations between serum neurofilament light chain (sNfL) levels at disease onset with markers of clinical severity in children with autoimmune encephalitis (AE). <h3>Background:</h3> Despite significant advances in the diagnosis and treatment of AE, little is known about markers of disease severity and their associations with outcomes. sNfL, a marker of axonal destruction, may fill this gap. <h3>Design/Methods:</h3> This is a longitudinal prospective case-control study of children presenting to a single tertiary center with AE (n=48) vs. an age-matched healthy control population (HC) (n=88). Clinical data and modified Rankin Scale (mRS) scores were collected using a standard CRF at baseline and at follow-up, up to one year. sNfL levels in stored samples were measured by Single Molecule Array Immunoassay (SIMOA). Volumetric analyses of key anatomic structures of brain MRIs were performed using FreeSurfer. Diffusion weighted MRI was assessed for restricted diffusion. Following standard diagnostic criteria, children were classified into those with no biological marker (antibody or genetic) and those with. We further dichotomized children into high vs. low sNFL levels. Descriptive statistics, group differences, and correlational analyses were performed using JASP version 0.16.3. <h3>Results:</h3> Baseline sNfL levels were significantly higher in patients with AE (no Ab), AE (MOG), and ANEC vs. HCs (log₂ sNfL=4.3 AE(no Ab), 2.9 AE(NMDA), 5.6 AE(MOG), 6.3(ANEC), 2.4(HC), <i>p</i>&lt;0.001). AE patients in the high sNfL group were more likely to be admitted to the ICU (<i>p</i>=0.02) and had significantly higher mRS scores at nadir (<i>p</i>=0.04), compared to patients in the low sNfL group. However, there was no significant difference in mRS scores at follow-up between groups. Baseline sNfL correlated significantly with baseline restricted diffusion (r_s(43)=0.45, <i>p</i>=0.002) and thalamic volumes (r_s(31)=0.45, <i>p</i>=0.009). <h3>Conclusions:</h3> Baseline sNfL levels may serve as a marker of disease severity in patients with AE. Future studies are needed to evaluate their associations with long-term outcomes. <b>Disclosure:</b> Ms. Da Silva has nothing to disclose. Ms. Yea has nothing to disclose. Dr. AlYanbuaawy has nothing to disclose. Dr. Matt has nothing to disclose. Dr. Longoni has nothing to disclose. Helen Branson has nothing to disclose. Logi Vidarsson has nothing to disclose. An immediate family member of Dr. Ertl-Wagner has received personal compensation for serving as an employee of Siemens Healthineers. Dr. Yeh has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Yeh has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for PRIME. Dr. Yeh has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis. Dr. Yeh has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. The institution of Dr. Yeh has received research support from Biogen. The institution of Dr. Yeh has received research support from Stem Cell Network. The institution of Dr. Yeh has received research support from CIHR. The institution of Dr. Yeh has received research support from Ontario Institute for Regenerative Medicine. The institution of Dr. Yeh has received research support from National MS Society. The institution of Dr. Yeh has received research support from NIH. The institution of Dr. Yeh has received research support from SickKids Foundation. The institution of Dr. Yeh has received research support from CMSC. The institution of Dr. Yeh has received research support from MSSC.

A Tale of Two Cases: When to Use PLEX That is The Question (P10-5.025)

<h3>Objective:</h3> NA <h3>Background:</h3> The diagnosis New Onset Status Epilepticus (NORSE) is rare. Autoimmune encephalitis (AIE) is one of the most common causes. Data on timely administration of Plasmapheresis (PLEX) vs IVIG/Solumedrol therapy is lacking. Seizure control can be used as a marker for response to therapy. <h3>Design/Methods:</h3> NA <h3>Results:</h3> In these two cases, we describe NORSE with newly diagnosed Autoimmune encephalitis. The clinical courses for each were different. Case 1, a 30 yo M with no pmh was admitted to Epilepsy Monitoring Unit (EMU). Patient was in focal status with LP CSF findings consistent with AIE. Patient had conventional treatment for AIE: steroids and IVIG. Patient was discharged upon clinical improvement. He was admitted a week later for worsening seizures. Patient’s seizures were treated with 5 PLEX sessions followed by chemotherapy (Actemra and Rituxan) administration in addition to high doses of antiseizure medication. Case 2, 32 yo M with no pmh presents with 11 days of generalized weakness, cognitive slowing/confusion. Patient had 6 generalized tonic clonic seizures. Patient was in the ICU and intubated. Various agents were used for seizure control such as Vimpat, Keppra, Depakote and Ketamine. Day 4, was started on steroids and IVIG. Day 11, started on 5 sessions of PLEX followed by chemotherapy (Actemra and Rituxan) with significant improvement. <h3>Conclusions:</h3> NORSE is a diagnosis with high rate of refractoriness to treatment and severe outcome. Early initiation of immunological therapy provide the most benefit. First line therapy is based on clinical presentation and expert opinion. These two cases illustrate better outcome with early initiation of plasmapheresis in rapidly progressing refractory focal status epilepticus of autoimmune etiology. Its been shown PLEX is better economically and less potential side effects as compared to IVIG. Rapid seizure control with combination of high dose new anti-seizure and immunological treatment in a timely manner are key for better outcomes. <b>Disclosure:</b> Dr. Dalal has nothing to disclose. Dr. Grunstein has nothing to disclose. Dr. Schneider has nothing to disclose. Dr. Proteasa has nothing to disclose.

The therapeutic effect of ofatumumab in autoimmune encephalitis: A case series.

The management of autoimmune encephalitis (AE) with immunotherapy is non-standardized, especially in refractory AE. Ofatumumab (OFA), an anti-CD20 antibody, has not been reported in the treatment of AE. This study presented three AE cases that received the OFA treatment. OFA was administered subcutaneously at a dose of 20mg two or three times within three weeks. There were some mild adverse effects, including low-grade fever and dizziness. They had favorable responses (reduced antibody titer and clinical symptom improvement). Their symptoms were stable and even improved during a three-month follow-up. Thus, OFA injection is demonstrated to be safe and effective in treating AE. This is the first report about OFA treatment in AE, depicting its potential as a therapeutic option.

Autoimmune Encephalitis: A Case Report from the Neurology Department of the Doctor Joseph Guislain Neuropsychiatric Centre in Lubumbashi and Review of the Literature

The term encephalitis refers to the inflammation of the parenchyma of the central nervous system, located in the cranial cavity. We report the case of a 17-year-old female patient with a history of tuberculous gonarthritis, who was admitted for convulsive seizures complicated by status epilepticus. Medical imaging did not reveal any brain lesions and biology revealed an elevated PCT. Through this case, we underline the risk of diagnostic errancy and thus of delay in the treatment of autoimmune encephalitis which, in 70% of cases, is effective. It also seemed important to us to insist on the fact that the absence of autoantibodies does not invalidate the diagnosis of autoimmune encephalitis, especially in developing countries where the technical platform to search for them is lacking both in terms of equipment and human resources. Finally, it should be noted that new autoantibodies are found almost every year by teams in developed countries. Thus, in front of any acute or sub-acute neurological picture not explained, it is necessary to think about autoimmune encephalitis and to start an immunomodulatory treatment, which guarantees the cure or the substantial improvement in most cases.

A Rare Case of Anti-N-Methyl-D-Aspartate Receptor Encephalitis in an Infant Presenting with Regression and Movement Disorder.

A Rare Case of Anti-N-Methyl-D-Aspartate Receptor Encephalitis in an Infant Presenting with Regression and Movement Disorder.

Autoimmune Encephalitis with Autoantibodies to NMDAR1 following Herpes Encephalitis in Children and Adolescents.

Herpes simplex virus (HSV) type 1 is a frequent pathogen causing infectious encephalitis (HSVE). Early treatment with intravenous acyclovir has led to a significant decrease in mortality. However, especially in children, deterioration during or after HSVE may occur without any evidence of HSV reactivation or improvement following repeated antiviral therapy. Here, we report 15 patients (age range 3 months to 15 years) who suffered from autoimmune encephalitis with autoantibodies to NMDAR1 following Herpes encephalitis, presenting with movement abnormalities (young children) or neuropsychiatric symptoms (older children) as major complaints, respectively. The diagnosis was based on positive cerebrospinal fluid (CSF) and/or serum anti-NMDAR-antibodies with two children showing only positive CSF antibody findings. The time lag between first symptoms and diagnosis of autoimmune encephalitis was significantly longer than between first symptoms and diagnosis of HSVE (p <0.01). All patients improved during immunosuppressive treatment, during which plasmapheresis or rituximab treatments were applied in 11 patients, irrespective of their age. Despite immunotherapy, no patients relapsed with HSVE. Early diagnosis and treatment of autoimmune encephalitis after HSVE may be associated with a better outcome so that high clinical awareness and routine testing for anti-NMDAR-antibodies after HSVE seems advisable. If autoimmune encephalitis is suspected, antibody testing should also be performed on CSF if negative in serum.

Evaluation of multiple consensus criteria for autoimmune encephalitis and temporal analysis of symptoms in a pediatric encephalitis cohort.

ObjectiveTo evaluate the sensitivity and specificity of current criteria for the diagnosis of autoimmune encephalitis (AE) and the temporal onset of neuropsychiatric symptoms (NP) in a pediatric encephalitis cohort.BackgroundMultiple criteria for AE have been developed, including the Graus and pediatric-focused Cellucci consensus criteria, and the Determining Etiology in Encephalitis (DEE) score for patients with encephalitis. Early identification and treatment of AE is crucial to improve outcomes, but this can be difficult given the frequent overlap of clinical presentation between AE and infectious encephalitis (IE).Design/methodsA retrospective review was conducted of patients seen at our institution from 2000 to 2021 with a final diagnosis of AE or IE. These were narrowed through multiple exclusions to etiology-confirmed IE or antibody-positive/negative AE. Time of onset or results of all symptoms and diagnostics were recorded. Sensitivity and specificity of each criterion under various clinical scenarios were calculated over the first month after initial NP symptom onset.ResultsA total of 23 antibody-positive AE, 9 antibody-negative AE and 23 IE patients were included in final analysis. Under an idealized scenario with rapid initial diagnostic evaluations, the sensitivity for pediatric AE by day 28 after onset of NP symptoms approached 90% for both Cellucci and Graus criteria. Specificity within these 28 days was low without infectious testing results, increasing the greatest with rapid PCR testing and second with infectious antibody testing—reaching ~90% with both. A DEE score of 3 provided a specificity of 100% in identifying IE, but low sensitivity (29%). Symptoms were noted to cluster within several days of onset in IE, but in AE were spread out. Personality/behavioral change, speech change, affective disorder, and sleep disturbance were noted more often in AE, while fever, elevated C-reactive protein or CSF protein, and abnormal MRI-Brain occurred more often in IE.ConclusionIn this study, we provide the first evaluation of the Cellucci criteria and the first validation of the DEE score in the differentiation of pediatric AE and IE. Further refinement of AE criteria is needed to improve early detection and treatment of pediatric AE.

Anti-NMDAR Encephalitis with Poor Recovery on Steroid Pulse and IVIg: Practical Approach to Intensive Immunotherapy

Anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) is a well-defined autoimmune encephalitis that is responsive to early intensive immunotherapy. Recent international consensus regarding treatment of NMDARE provides a practical treatment algorithm for immunotherapy escalation, while considering a patient's age, disease severity, and other background information. First-line immunotherapy, which includes an intravenous (IV) corticosteroid pulse with the addition of either intravenous immunoglobulins (IVIg) or plasma exchange, should be offered to all NMDARE-diagnosed patients as soon as possible. In cases where insufficient improvement follows a repeat of the first-line combination therapy (assessed on day 14 after the initial treatment), second-line immunotherapy comprising rituximab or an IV cyclophosphamide pulse (IVCPA) is considered. Per the recent expert consensus, rituximab is preferred to IVCPA as the second-line drug of choice, although the use of either drug in the treatment of NMDARE is off-label. Most patients show gradual improvement in the first few weeks following the introduction of second-line therapy, although repeated and alternating use of both drugs is often required. Some patients, whose NMDARE was refractory to the aforementioned therapies, have also been successfully treated with tocilizumab or bortezomib. Moreover, several international clinical trials involving rituximab, inebilizumab, bortezomib, and rozanolixizumab in the treatment of autoimmune encephalitis (AE, which includes NMDARE) are being conducted to establish high-grade evidence for the treatment of AE.

Brain Perfusion Alterations on 3D Pseudocontinuous Arterial Spin-Labeling MR Imaging in Patients with Autoimmune Encephalitis: A Case Series and Literature Review.

Autoimmune encephalitis is a heterogeneous group of newly identified disorders that are being diagnosed with increasing frequency. Early recognition and treatment of autoimmune encephalitis are crucial for patients, but diagnosis remains challenging and time-consuming. In this retrospective case series, we describe the findings of conventional MR imaging and 3D pseudocontinuous arterial spin-labeling in patients with autoimmune encephalitis confirmed by antibody testing. All patients with autoimmune encephalitis showed increased CBF in the affected area, even when some of them presented with normal or slightly abnormal findings on conventional MR imaging. Additionally, serial 3D pseudocontinuous arterial spin-labeling showed perfusion reduction in 1 patient after therapy. For patients with highly suspected autoimmune encephalitis, 3D pseudocontinuous arterial spin-labeling may be added to the clinical work-up. Further studies and longitudinal data are needed to corroborate whether and to what extent 3D pseudocontinuous arterial spin-labeling improves the diagnostic work-up in patients with autoimmune encephalitis compared with conventional MR imaging.