Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARE) is a complex, yet treatable autoimmune disorder characterized by a fairly abrupt onset of a constellation of symptoms attributable to diffuse brain dysfunction (Tarantino et al., 2021). Despite the potential for a severe disease course, most patients have a favorable outcome with substantial recovery (Dalmau et al., 2011; Titulaer et al., 2013). Nevertheless, there is limited literature discussing the long-term outcomes in patients with anti-NMDARE, particularly in pediatric patients. The primary objective of this study is to examine and describe behavioral, emotional, adaptive, and executive functioning outcomes in pediatric and young adult patients with this disease. This study also sought to provide information on the perceived health-related quality of life (HRQoL) of patients and their parents and investigate the impact of anti-NMDARE on parents and family functioning.

All individuals known to have been diagnosed and treated for anti-NMDARE at The Children’s Hospital of Philadelphia (CHOP) between January 1, 2005, and October 1, 2020, were contacted with both patients and their parents/guardians invited to participate. Eighteen pediatric patients between the ages of 6 and 26 and/or their parents/caregivers participated in the study. Of the 18 patients represented in the sample, 50% were white/Caucasian, and 67% were female. The mean duration of time since symptom onset was 7.1 years. Primary outcomes were measured through standardized questionnaires of emotional, behavioral, and adaptive functioning (BASC-3) and executive functioning (BRIEF2 or BRIEF-A). Secondary outcomes related to family functioning and HRQoL were measured through (PedsQL™ and PedsQL™ Family Impact Module.)

All aggregate T-scores for the BASC and BRIEF placed children with anti-NMDARE within an age-appropriate range regarding behavioral, emotional, adaptive, and executive functioning outcomes. Children with anti-NMDARE were not found to have lower HRQoL compared to their healthy same-age peers. Moreover, parents of children with anti-NMDARE did not endorse a prolonged impact of this illness on family functioning and adjustment.

This study aimed to better understand the neurobehavioral profile and the long-term outcomes of children diagnosed with anti-NMDARE, with the ultimate goal of advancing understanding of this encephalitis. Consistent with findings from several reviewed studies on long-term follow-up, the present study suggests that most children with a history of anti-NMDARE show good functional recovery over time. However, data on the neurobehavioral sequelae, quality of life, and adaptive behavior in patients diagnosed with anti-NMDARE are still sparse, especially at pediatric age. In order to understand and learn to manage the needs of patients with anti-NMDARE, particularly regarding the impact this disease can have on daily life and school performance, additional neuropsychological research involving larger samples, longitudinal studies, and increased methodological consistency is required.

Early and effective treatment of central nervous system (CNS) inflammatory disorders is vital to reduce neurologic morbidity and improve long-term outcomes in affected children. Rituximab is a B-cell-depleting monoclonal antibody whose off-label use for these disorders is funded in the province of Alberta, Canada, by the Short-Term Exceptional Drug Therapy (STEDT) program. This study describes the use of rituximab for pediatric CNS inflammatory disorders in Alberta.

Rituximab applications for CNS inflammatory indications in patients <18 years of age were identified from the STEDT database between January 1, 2012, and December 31, 2019. Patient information was linked to other provincial datasets including the Discharge Abstract Database, Pharmaceutical Information Network, and Provincial Laboratory data. Analysis was descriptive.

Fifty-one unique rituximab applications were identified, of which 50 were approved. New applications increased from one in 2012 to a high of 12 in 2018. The most common indication was autoimmune encephalitis without a specified antibody (n = 16, 31%). Most children were approved for a two-dose (n = 33, 66%) or four-dose (n = 16, 32%) induction regimen. Physician-reported outcomes were available for 24 patients, of whom 14 (58%) were felt to have fully met outcome targets.

The use of rituximab for pediatric CNS inflammatory disorders has increased, particularly for the indication of autoimmune encephalitis. This study identified significant heterogeneity in dosing practices and laboratory monitoring. Standardized protocols for the use of rituximab in these disorders and more robust outcome reporting will help better define the safety and efficacy of rituximab in this population

Anti-Hu related Paraneoplastic Neurological Syndrome (PNS) is one of the most common paraneoplastic-associated neurological syndromes (Kayser 2010). While the primary clinical manifestations are neurologic in nature (Smitt 2002), only rare reports exist regarding psychiatric manifestations. Our poster presents two cases of Anti-Hu Encephalitis manifesting as psychosis as well as a systematic literature review on the co-occurrence of psychosis and PNS.

The aim of this case series is to show psychosis as the primary symptom of a paraneoplastic syndrome that does not typically present in this way. It also serves as a reminder to have a detailed work-up and maintain a wide differential diagnosis when evaluating patients with first-episode psychosis.

Two cases of anti-Hu encephalitis primarily presenting with psychiatric symptoms are discussed. A systematic literature review was carried out based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) model on three electronic databases: PubMed, Embase, and PsycINFO. Search terms included were (Anti-Hu) AND (Psychosis OR Hallucinations OR Schizophrenia OR Schizoaffective).

Our case series reports on two patients with diagnosed anti-Hu encephalitis who were treated by our psychiatry team, where the primary manifestations of the illness were psychiatric in nature. Psychotic symptoms in these cases were managed with Risperidone, Olanzapine, and Paliperidone.

Psychotic symptoms are seldom reported in the literature and cases like the ones presented emphasize the importance of a full medical work-up for first episode psychosis as well as a wide differential. Given the increased association between PNS and psychiatric illness, more emphasis and further research is warranted.

No significant relationships.

Autoimmune encephalitis are inflammatory diseases of the CNS mediated by antibodies that attack neurotransmitter receptors or proteins on the surface of neurons, usually in the limbic system. The clinic is different according to the antineuronal Ac involved.

To make a correct differential diagnosis between autoimmune encephalitis and primary psychiatric pathologies that may be similar in symptoms through a complete study of the patient including anamnesis, physical examination, imaging tests, cerebrospinal fluid and serum studies.

Description of a clinical case. A 31-year-old female patient, with no previous history of interest, was brought to the emergency department for a suspected seizure. The previous days she had presented emotional lability, difficulty in concentration and reading, blurred vision, confusion and hemicranial headache. Two days later she returned to the emergency room for insomnia, dysarthria, difficulty in reading, comprehension, naming, and excessive rumination of her problems. Incoherent and repetitive language. The Emergency service requested to rule out a conversive disorder.

Neuropsychiatric manifestations (anxiety, depression, behavioral disturbances, insomnia, memory deficits, psychomotor agitation, mania, auditory and visual hallucinations, delusions) are the first symptom in 70% of autoimmune encephalitis due to anti-NMDA antibodies and usually respond poorly to psychiatric treatment, making the treatment of the primary cause necessary for the remission of these symptoms.

Given their increasing recognition and prevalence, autoimmune causes should always be taken into account in behavioral changes, cognitive or consciousness impairment of subacute installation, especially in young patients and once infectious, metabolic and vascular causes have been ruled out with an appropriate complementary study.

No significant relationships.

Anti-NMDA receptor encephalitis is a disease occurring when antibodies produced by the body’s own immune system attack NMDA receptors in the brain. Their functions are critical for judgement, perception of reality, human interaction, the formation and retrieval of memory, and the control of autonomic functions. The objective of treatment is to reduce the levels of antibodies in the blood and spinal fluid. Treatments include corticosteroids, intravenous immunoglobulin and plasmapheresis in addition to other immunomodulators, such as cyclophosphamide or rituximab.

To present a case of a 64 year-old patient who came to the emergency service of our hospital with long-standing anxiety, irritability, recurrent amnestic failures, visual hallucinations and recent-onset episodes of aggressiveness with his family. He required admission to the psychiatry department and was finally diagnosed with autoimmune anti-NMDA encephalitis by detecting antibodies in blood and CSF.

Clinical case presentation and literature review of cases, focusing on psychotic symptoms.

A 65-year-old patient who was being studied by neurology and psychiatry departments for cognitive impairment and psychotic symptoms was admitted to Neurology after a positive lumbar puncture result for NMDA antibodies.During admission, the patient continued with a significant behavioral alteration that gradually remitted with the use of Quetiapine, corticosteroids and rituximab.

NMDA-encephalitis has a highly variable clinical presentation, which can lead to confusion with infectious etiology or psychiatric disorders, making the diagnosis difficult, which is only possible by detecting anti-NMDA antibodies in CSF. Recognition of the disease and coordination between services is essential for early diagnosis and treatment.

No significant relationships.

Anti-N-methyl-D-aspartate receptor (anti-NMDA-R) encephalitis is well-characterised autoimmune encephalitis with prominent psychiatric manifestations, neurological manifestations like speech dysfunction, seizures, dyskinesias and other movement abnormalities, decreased level of consciousness and autonomic instability. This disorder affects primarily children and adults up to 45 years. Females are 4 times more common than males and may have association with ovarian teratoma.

To identify anti-NMDA receptor encephalitis based on clinical features, facilitate early screening and relevant investigations to prevent delay in treatment.

A case study of 36 year old female presented with clinical manifestations of autoimmune encephalitis syndrome.

Diagnosis confirmed by presence of NMDA receptor antibodies in serum and cerebrospinal fluid.

Early recognition of clinical features of Anti-NMDA receptor encephalitis and early initiation of treatment has shown to improve outcomes, speed recovery and reduce the risk of relapses.

No significant relationships.

Albeit primarily a disease of respiratory tract, the 2019 coronavirus infectious disease (COVID-19) has been found to have causal association with a plethora of neurological, neuropsychiatric and psychological effects. This review aims to analyze them with a discussion of evolving therapeutic recommendations.

PubMed and Google Scholar were searched from 1 January 2020 to 30 May 2020 with the following key terms: “COVID-19”, “SARS-CoV-2”, “pandemic”, “neuro-COVID”, “stroke-COVID”, “epilepsy-COVID”, “COVID-encephalopathy”, “SARS-CoV-2-encephalitis”, “SARS-CoV-2-rhabdomyolysis”, “COVID-demyelinating disease”, “neurological manifestations”, “psychosocial manifestations”, “treatment recommendations”, “COVID-19 and therapeutic changes”, “psychiatry”, “marginalised”, “telemedicine”, “mental health”, “quarantine”, “infodemic” and “social media”. A few newspaper reports related to COVID-19 and psychosocial impacts have also been added as per context.

Neurological and neuropsychiatric manifestations of COVID-19 are abundant. Clinical features of both central and peripheral nervous system involvement are evident. These have been categorically analyzed briefly with literature support. Most of the psychological effects are secondary to pandemic-associated regulatory, socioeconomic and psychosocial changes.

Neurological and neuropsychiatric manifestations of this disease are only beginning to unravel. This demands a wide index of suspicion for prompt diagnosis of SARS-CoV-2 to prevent further complications and mortality.