Autoantibodies Targeting Unknown Epitope Autoimmune Encephalitis and the Effect of Modified Electroconvulsive Therapy: Report of Three Cases.

Objective: Generally autoimmune encephalitis (AE) cases present with central nervous system symptoms. Many types of autoantibodies are associated with autoimmune encephalitis, with anti-N-methyl-D-aspartate receptor being the most commonly reported. However, autoimmune encephalitis cases with autoantibodies targeting unknown epitopes are increasingly recognized. This article aims to summarize the clinical experience and assess the feasibility of modified electroconvulsive therapy (MECT) as an adjunctive treatment method for autoimmune encephalitis patients with poor response to first-line immunotherapy and mainly displaying psychiatric symptoms. Methods: This work reports three cases of which two have been diagnosed as autoantibodies targeting unknown epitope autoimmune encephalitis while one has been diagnosed as anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and all were effectively treated with MECT. Results: All three cases that otherwise failed to respond to standard immunotherapy for controlling psychiatric symptoms exhibited excellent clinical outcomes following MECT. The underlying mechanism of action of MECT is unclear and whether such an effect involves a neurotransmitter rebalance in the brain remains uncertain. At present, we have observed only a small number of clinical cases, warranting further research among a larger number of clinical cases and more systematic multicenter retrospective analysis. Conclusions: It should be noted that, while our experience supports the utility of MECT in the treatment of certain cases of AE, this option should be regarded as an adjuvant therapy after standard immunosuppressive therapy. Clinicians must be aware that patients should be provided with psychiatric or neurological services for timely diagnosis along with timely and appropriate treatment.

[The Exorcist, The 8-Year Engagement, and Brain on Fire: Three Films about Anti-N-methyl-D-aspartate Receptor Encephalitis].

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is one of the most common forms of autoimmune encephalitis. It is more prevalent in young adult women than it is in men. The disease is characterized by a rapid progression of psychiatric symptoms and/or cognitive impairment. Common symptoms include changes in behavior (i.e. hallucination, paranoia, and aggression etc.), seizures, memory deficit, abnormal movements, and coma. Based on these symptoms, it was historically thought that this 'behavior' was the work of the devil, and exorcism by a shaman was sometimes required. In line with this, it has been suggested that the patient who the original Exorcist movie was based on had this disease. Although immunotherapy is an effective treatment for anti-NMDA encephalitis, recovery is slow with the majority of patients requiring more than a year after disease onset to make a full recovery. In light of this, the stories and experiences of patients with this disease have been depicted in films. Examples of the films include: The 8-year engagement (2017), a Japanese film directed by Takahisa Zeze, and Brain on fire (2016), directed by Irish filmmaker Gerrard Barrett. These films help to raise awareness and make this disease more recognized by the general public.

A case of GFAP-IgG positivity followed by anti-NMDAR encephalitis.

BACKGROUND: In recent years, there have been an increasing number of reports on overlapping antibodies in autoimmune encephalitis (AE). There are various types of overlapping antibodies, but the clinical significance of each type is not yet clear. Glial antibodies, such as MOG, AQP4, and especially NMDAR, can be detected in patients with AE. However, little is known about the overlapping antibodies of anti-glial fibrillary acidic protein (GFAP), and only a few case reports have described this overlap. Case presentation The patient was a 7-year-old girl with recurrent intermittent fever and seizures, and viral encephalitis was diagnosed at the beginning of the disease. She was discharged after treatment with acyclovir, high-dose immunoglobulins, and valproic acid as an antiseizure medication. Subsequently, the patient still had occasional seizures and abnormal behavior, and the anti-NMDAR antibody test was positive (1:3.2). She was treated with high-dose methylprednisolone and antiseizure therapy. Approximately half a year later, the patient experienced fever and seizures again, serum GFAP IgG was 1:100, and a head MRI indicated new lesions. Improvement was achieved after repeated high-dose methylprednisolone and continuous prednisone anti-inflammatory therapy. CONCLUSIONS: Anti-NMDAR encephalitis combined with GFAP-IgG is uncommon, and repeated tests for AE-associated antibodies may be required in patients with recurrent encephalitis. Compared with cerebrospinal fluid antibody-positive children, serum GFAP IgG-positive children should be comprehensively diagnosed according to their clinical manifestations. It is worth considering whether overlapping antibody syndrome can still be an issue for patients with AE who recover and have negative antibodies after a few months if disease recurrence and new antibodies are detected.

The Challenge of Integrating Care in Dual Diagnosis; Anti-NMDA-Receptor Encephalitis; Presentation And Outcome In 3 Cases Referred For Complex Specialist Rehabilitation Services.

The successful implementation of an integrated care pathway (ICP) for any given condition is a challenge. Even more challenging is successful ICP implementation for individuals who have multiple co-morbidities. This is further compounded when there are dual mental health and physical disabilities that require integrated working across multiple disciplines, specialties, institutions and organisations. Anti-NMDA-Receptor encephalitis (aNMDARe) is a relatively new diagnostic entity with patients typically presenting with significant psychiatric symptoms followed by progressive neurological deterioration. In this case series, we describe 3 cases of females with aNMDARe who were referred for complex specialist rehabilitation (CSR) to The National Rehabilitation Hospital. CSR is the total active care of patients with a disabling condition, and their families, by a multi-professional team who have undergone recognised specialist training in rehabilitation, led /supported by a consultant trained and accredited in rehabilitation medicine (RM). These services provide for patients with highly complex rehabilitation needs that are beyond the scope of local services. In these cases, referral to CSR resulted in the construction of a bespoke integrated care pathway (ICP) that transcended the barriers between primary, secondary and tertiary care and across the boundaries of physical and mental health. A care pathway is a complex intervention for the mutual decision-making and organisation of care processes Rehabilitation services acted as the coordinator of services in these cases to ensure implementation of the care plan and to ensure successful transitions of care and supported local specialist and general teams in the management of these complex cases.

[Workup and treatment of autoimmune encephalitis]. / Udredning og behandling afautoimmun encephalitis.

Autoimmune encephalitis with antibodies against neuronal surface antigens is diagnosed with increasing frequency in recent years. If treated early and aggressively, these conditions often respond favourably to immunotherapy. We describe the clinical features, diagnosis and treatment of the two most common types of autoimmune encephalitis with antibodies against the N-methyl-D-aspartate receptor or the leucine-rich glioma-inactivated 1 protein. Together, these two conditions comprise 80% of the autoimmune encephalitis cases diagnosed in Denmark. Autoimmune encephalitides with rare antibodies are also summarized.

Anti-NMDA receptor encephalitis: psychiatric presentation and diagnostic challenges from psychosomatic medicine perspective.

We describe two cases of confirmed anti-NMDA receptor encephalitis; one patient initially presented with a clinical picture that resembled delirium and later appeared to present with a conversion reaction and the second patient presented with a first psychotic break followed by the clinical picture of neuroleptic malignant syndrome with catatonia. Neither patient had a previous history of psychiatric illness or recreational drug use. These cases illustrate the diagnostic and treatment challenges associated with this neuropsychiatric condition and underscore the role of psychosomatic medicine psychiatrists in diagnosing anti-NMDA receptor encephalitis.