Autoimmune Encephalitis Presenting Research Articles

Treatment of Anti-GAD65 Autoimmune Encephalitis Presenting as NORSE with Intrathecal Dexamethasone (P1-14.005)

Treatment of Anti-GAD65 Autoimmune Encephalitis Presenting as NORSE with Intrathecal Dexamethasone (P1-14.005)

Occult Lung Cancer-Associated Autoimmune Encephalitis Presenting as Acute Psychosis.

During deployment, a 52-year-old male developed acute behavioral changes. Though initially considered to have PTSD and related agitation and confusional state, his evaluation was consistent with acute encephalopathy. Magnetic resonance imaging of the brain showed T2 hyperintensities, and CSF analysis was positive for anti-N-methyl-D-aspartate receptor antibody. A nuclear protein in testis carcinoma midline carcinoma was discovered in the lung. Immunotherapy and surgical resection led to steady improvement prior to adjuvant chemotherapy. Autoimmune encephalitis due to anti-N-methyl-D-aspartate receptor antibodies is increasingly being recognized as causal of acute behavioral change.

Catatonia as a Presentation of Autoimmune Encephalitis: A Systematic Review and Meta-analysis

Catatonia as a Presentation of Autoimmune Encephalitis: A Systematic Review and Meta-analysis

Autoimmune encephalitis presenting with atypical parkinsonism: A case report and review of the literature

AbstractAn 81‐year‐old man developed axial rigidity, bradykinesia, and cognitive impairment within 6 weeks. On initial examination, he was misdiagnosed with progressive supranuclear palsy (PSP). Brain magnetic resonance imaging showed hyperintensities in the bilateral mesial temporal lobes and basal ganglia. Neuronal antibodies previously reported in autoimmune encephalitis mimicking PSP were negative. Immunohistochemical and immunocytochemical analysis revealed the autoantibodies recognized neuronal surface and intracellular antigens. The diagnostic criteria for probable autoimmune encephalitis were fulfilled. We diagnosed him with autoimmune encephalitis. Intravenous immunoglobulin and steroid therapy improved his symptoms. The presence of novel autoantibodies causing autoimmune encephalitis presenting with atypical parkinsonism was suggested.

Recurrent GRAF1 Antibody Encephalitis in Woman with History of Cancer and Powassan Encephalitis (P4-5.012)

<h3>Objective:</h3> To review details of a rare presentation of autoimmune encephalitis with antibody to GRAF1, which is rare and with little information about its origin or associated clinical syndrome, and educate clinicians to identify similar presentations early to allow effective treatment and prognostic counseling for patients and their family. <h3>Background:</h3> This case details presentation of a rare cause of autoimmune encephalitis with antibodies to GTPase regulator associated with focal adhesion kinase-1 (GRAF1). Reports in the literature may group this with other anti-Ca channel antibodies, and often associate it with an ataxic clinical syndrome. Little is known about the origin of these antibodies, as it is not always associated with malignancy or specific systemic conditions. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> A 68 year old woman with a history of breast and cervical cancer, as well as Powassan encephalitis had recurrent episodes of encephalitis with prominent confusion, and was found to have a high titer of antibody to GRAF1 in her CSF with each clinical episode. She unfortunately did not respond well to immunosuppressive medications or IVIG, and passed away on hospice. The details of this case report highlight the clinical features associated with this case of GRAF1 antibody encephalitis and poses questions about whether cancer or prior CNS infection may be the origin of these antibodies, as our patient had a history or Powassan encephalitis. It is also not clear whether serial CSF testing can be used as maintenance to guide management. <h3>Conclusions:</h3> GRAF1 antibody encephalitis is a poorly understood cause of autoimmune encephalitis, without a consistent associated clinical syndrome, or associated condition that may be a source of the increased antibody titer. We pose the question whether this can be a post-infectious entity, and highlight a case of encephalitis which had recurrent episodes before becoming progressive and refractory to treatment. <b>Disclosure:</b> Dr. Dobson has nothing to disclose. Dr. Crissinger has nothing to disclose.

A rare case of autoimmune encephalitis presenting as first psychotic episode

A rare case of autoimmune encephalitis presenting as first psychotic episode

Rapidly Progressive Dementia With Recurrent Seizures and Hyponatremia; A Case of LGI1 Limbic Encephalitis

Objective N/A. Background Leucine-Rich Glioma Inactivated Protein-1 (LGI1) autoimmune encephalitis was first described in 2001 as one of the syndromes caused antibodies against the voltage-gated potassium channels (VGKC) until it was discovered in 2010 that antibodies were instead being directed towards the protein LGI1. This often presents in males in their 60's and is often associated with faciobrachial dystonic seizures, which have become path pneumonic for this disease process. Design/Methods N/A. Results 77-year-old female with history of hyponatremia, anxiety, hypertension, and lacunar infarct presented for a concern for seizures. She presented for multiple episodes of reported generalized tonic seizures and was eventually found to have right frontotemporal seizures with impaired awareness. Magnetic Resonance Imaging (MRI) was repeated multiple times but were significantly degraded due to motion artifact and read as limited. Further discussion with husband was concerning for memory loss over the past 4 months, but patients children disputed this with several years of memory loss. After neuropsychological testing which demonstrated significant decline across multiple domains. MRI was revisited which was concerning for bilateral mesial temporal hyperintensities on Fluid-Attenuated Inversion Recovery (FLAIR). Patient underwent lumbar puncture given unremarkable workup thus far. CSF and serum both demonstrated LGI1 autoantibodies for which the patient received a 5 days course of IV methylprednisolone, IV immunoglobulins, and was eventually transitioned to rituximab with complete recovery of long term memory. Conclusions This case demonstrates the complexity of evaluating a patient for reported rapidly progressive dementia and some of the pitfalls involved in the workup. This case demonstrates that when the initial workup is unremarkable, the patient should be evaluated for uncommon causes, such as autoimmune encephalitis. We diagnosed an atypical presentation of autoimmune encephalitis and documented the initial treatment and response to both first line and second line treatment with future plans to titrate the anti-epileptic drugs.

(34) COVID-19 Auto-Immune Encephalitis presenting as First-Break Psychosis: A Case Report and Literature Review

(34) COVID-19 Auto-Immune Encephalitis presenting as First-Break Psychosis: A Case Report and Literature Review

Rare autoimmune encephalitis presenting as fluid-attenuated inversion recovery-hyperintense lesions in anti-Myelin oligodendrocyte glycoprotein-associated encephalitis and seizures accompanied with anti-IgLON5 antibody.

Rare autoimmune encephalitis presenting as fluid-attenuated inversion recovery-hyperintense lesions in anti-Myelin oligodendrocyte glycoprotein-associated encephalitis and seizures accompanied with anti-IgLON5 antibody

Autoimmune Encephalitis Presenting as Dystonia-Parkinsonism

Autoimmune encephalitis refers to a variety of conditions in which the body's immune system attacks the brain. The disease may present acutely but can also persist through a chronic progressive or relapsing-remitting time course.[1][1] The pathology of autoimmune encephalitis is either antibody- or

Autoimmune encephalitis presenting as a psychiatric disorder: A diagnostic challenge

Sri Lanka Journal of Medicine is the official journal of the Kandy Society of Medicine. It is a peer-reviewed journal currently published biannually. Original articles, reviews, brief reports, case reports, points of view, and letters to the editor, in all fields of medical science and practice are accepted for publication.The Sri Lanka Journal of Medicine is now indexed in DOAJ

Immuno-mediated movement disorders

Movement disorders may be the first or most prominent presentation of autoimmune encephalitis. They may present with characteristic phenotypes, have associated red flags or other clues to the diagnosis. Importantly, they may also be a differential diagnosis of degenerative disease, particularly when signs and symptoms develop slowly. The talk gives an overview and an update of the broad clinical spectrum of movement disorders with neuronal antibodies and autoimmune movement disorder mimics, highlighting red flags and clinical clues to guide neurologists in clinical practise.

Probable autoimmune encephalitis presenting with epilepsy, fever and ageusia: A possible COVID-19-related event?

Encephalitis is an acute inflammation of brain parenchyma, clinically characterized by confusional state and cognitive decline, fever, headache, and an altered level of consciousness. Encephalitis is typically viral or autoimmune. Aim of the study is to discuss a case of autoimmune encephalitis with anosmia and elipeptic seizures at clinical presentation.

Encephalitis patient-derived monoclonal GABAA receptor antibodies cause epileptic seizures.

Autoantibodies targeting the GABAA receptor (GABAAR) hallmark an autoimmune encephalitis presenting with frequent seizures and psychomotor abnormalities. Their pathogenic role is still not well-defined, given the common overlap with further autoantibodies and the lack of patient-derived mAbs. Five GABAAR mAbs from cerebrospinal fluid cells bound to various epitopes involving the α1 and γ2 receptor subunits, with variable binding strength and partial competition. mAbs selectively reduced GABAergic currents in neuronal cultures without causing receptor internalization. Cerebroventricular infusion of GABAAR mAbs and Fab fragments into rodents induced a severe phenotype with seizures and increased mortality, reminiscent of encephalitis patients' symptoms. Our results demonstrate direct pathogenicity of autoantibodies on GABAARs independent of Fc-mediated effector functions and provide an animal model for GABAAR encephalitis. They further provide the scientific rationale for clinical treatments using antibody depletion and can serve as tools for the development of antibody-selective immunotherapies.

CASPR2 positive autoimmune encephalitis presenting as delirium in an alcohol dependent patient: a diagnostic dilemma

Delirium, a major psychiatric emergency commonly encountered in alcohol withdrawal state, can have various aetiologies. Here we present a case of protracted delirium in an alcohol dependent patient, which on detailed neurological workup was diagnosed to be autoimmune encephalitis with CASPR2 (contactin-associated protein-like 2) antibodies. Subsequent treatment with immunotherapy and steroids lead to the resolution of symptoms.

Orbitofrontal syndrome and “goosebumps”—A new manifestation of autoimmune epilepsy in anti-LGI1 encephalitis

Anti–leucine-rich glioma-inactivated 1 (anti-LGI1) encephalitis is a rare autoimmune disorder that presents with a triad of cognitive and psychiatric disturbances, hyponatremia, and autoimmune epilepsy. Facial-brachial dystonic (FBD) seizures are considered a pathognomonic sign of this condition. However, more subtle seizure types can occur and should be recognized, allowing for timely diagnosis and treatment. We report a case of anti-LGI1 autoimmune encephalitis presenting as orbitofrontal syndrome with focal “goosebump” seizures.

Autoimmune Encephalitis in Latin America: A Critical Review

Autoimmune encephalitis is an increasingly recognized cause of encephalitis. The majority of case series report patients residing in developed countries in the northern hemisphere. The epidemiologic features of autoimmune encephalitis in Latin America are still unclear. The aim of the study was to perform a review of the clinical presentation of autoimmune encephalitis in Latin America and compare to world literature. References were identified by an in-depth literature search and selected on the basis of relevance to the topic and authors' judgment. We selected clinical studies and case reports published from 2007 to July, 2020 including patients from Latin American countries. Of the 379 patients included, the majority were cases of anti-NMDA receptor encephalitis (93.14%), followed by anti-VGKC-complex encephalitis (N = 17; 4.48%), anti-GAD encephalitis (N = 9; 2.37%), anti-AMPA receptor encephalitis (N = 1; 0.26%), anti-GABA receptor encephalitis (N = 1; 0. 26%), anti-mGluR5 encephalitis (N = 1; 0. 26%), and anti-mGluR1 encephalitis (N = 1; 0. 26%). Reported cases of Anti-NMDA encephalitis in Latin-America had a very slight female predominance, lower prevalence of associated tumors and a lower incidence of extreme delta brush on electroencephalogram. Autoimmune encephalitis is possibly underdiagnosed in underdeveloped countries. Its outcome after treatment, however, appears to be similarly favorable in Latin American patients as has been reported in developed countries based on available case reports and case series. Regional specificities in the manifestation of autoimmune encephalitis could be related to epidemiologic factors, such as the presence of different triggers and different genetic and immunologic background, that need to be studied by future research.

Nonparaneoplastic anti-NMDA receptor autoimmune encephalitis presenting as psychosis: A case report.

Nonparaneoplastic anti-NMDA receptor autoimmune encephalitis presenting as psychosis: A case report.

Striational Muscle Antibody Positivity Heralding a Diagnosis of Autoimmune Encephalitis associated with Epiglottic Squamous Cell Carcinoma: A Case Report

A 59 yo woman presented to our emergency room with confusion, spells of loss of awareness over four weeks with malaise, weakness, gait difficulties, and 30 lb weight loss over one year. Brain imaging was unremarkable. EEGs revealed diffuse slowing, triphasic GPDs, and left temporal epilepti form discharges. Spinal fluid showed an elevated protein count. She received a working diagnosis of autoimmune encephalitis and received steroids and antiepileptic medications. She experienced an improvement in symptoms and went home. Follow up revealed elevated striational muscle antibodies and enhancement of spinal nerve roots suggesting an autoimmune neurological syndrome. She received intravenous immune globulin (IVIg) therapy and had periods of improvement followed by worsening a few months later with each dose of IVIg. She presented to the hospital a year with worsening dysphagia and an endoscopy showing an epiglottic mass. Pathology confirmed squamous cell carcinoma, and she received chemotherapy and radiation. We wish to share this rare case of autoimmune encephalitis presenting with only striational antibodies as a heralding sign of a tumor. We postulate that, in the correct context, the presence of striational antibodies alone, despite low titers, may support a diagnosis of autoimmune encephalitis from underlying malignancy.

Anti- NMDAR Autoimmune Encephalitis Presenting as Acute Psychosis: A Case Report

&#x0D; &#x0D; &#x0D; &#x0D; Anti-NMDA receptor (NMDAR) encephalitis is a recently identified autoimmune disorder with prominent psychiatric symptoms. Patients usually present with acute behavioral change, psychosis, catatonic symptoms, memory deficits, seizures, dyskinesias, and autonomic instability. We present a case of a 13-year old who presented with noticeably chirpiness, excessive talking with unknown people and wandering around the neighborhood without purpose.The main symptoms of the patient and the important clinical findings were irrelevant talking which later developed into slurring of speech, abnormal movements and memory loss.This case is an example of how easily we are misled towards diagnosis based on the present symptoms. The patient suffered the unnecessary stigma of a psychiatric illness, which might stay imprinted on her for a long time. In this report we call for attention to the inclusion of anti-NMDAR encephalitis in the differential diagnosis of acute psychosis. It adds on to show that NMDAR might present in the most unexpected and unpredictable ways, sometimes misleading the patient away from medical help.Prompt diagnosis is critical as early immunotherapy and tumor removal could dramatically affect outcomes.&#x0D; &#x0D; &#x0D; &#x0D;