A protocol for investigating the association of vaccination and anti-NMDA receptor encephalitis. Front Biosci (Schol Ed). 2018 Jan 01;10:229-237 Authors: Wang H Abstract Anti-N-methyl-D-aspartate (Anti-NMDA) receptor encephalitis is an acute autoimmune neurological disorder that can be triggered by virus, H1N1/tetanus/diphtheria/pertussis and polio vaccines or by presence of a...

Anti-NMDA receptor encephalitis is a distinct disorder characterised by the predictable sequential development of symptoms; prodromal symptoms are initially noted, followed by prominent psychiatric symptoms, seizures, an unresponsive/catatonic state, hypoventilation, and involuntary orofacial-limb movements. This disorder usually affects young women with ovarian teratoma but may also affect women of any age or even men. We report the case of a 32 year old primigravid woman who developed psychosis with associated catatonia and autonomic dysfunction at 8 weeks gestation. Cranial imaging in the form of CT and MRI was normal. EEG showed slow waves and anti-NMDA receptor encephalitis was suspected. This was confirmed by the finding of serum anti-NMDA antibodies. Transvaginal Ultrasound and pelvic MRI suggested normal ovaries. She required admission to the High Dependency Unit for several weeks but eventually responded to plasma exchange, steroids, azathioprine, Intravenous immunoglobulin and ...

TY - JOUR. T1 - Anti-N-methyl-d-aspartate receptor encephalitis with acute disseminated encephalomyelitis-like MRI features. AU - Lekoubou Looti, Alain Zingraff. AU - Viaccoz, A.. AU - Didelot, A.. AU - Anastasi, A.. AU - Marignier, R.. AU - Ducray, F.. AU - Rogemond, V.. AU - Honnorat, J.. PY - 2012/2/1. Y1 - 2012/2/1. UR - http://www.scopus.com/inward/record.url?scp=84856031465&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=84856031465&partnerID=8YFLogxK. U2 - 10.1111/j.1468-1331.2011.03617.x. DO - 10.1111/j.1468-1331.2011.03617.x. M3 - Letter. C2 - 22182357. AN - SCOPUS:84856031465. VL - 19. JO - European Journal of Neurology. JF - European Journal of Neurology. SN - 1351-5101. IS - 2. ER - ...

Pearls Anti-NMDA receptor encephalitis can coexist with an overlapping demyelinating syndrome. An atypical presentation of a single autoimmune disorder should prompt investigation for coexistent autoimmune disorders. Discovery of overlap syndromes is important because the management and prognosis may be different. Oysters In autoimmune encephalitis, shorter time from symptom onset to treatment initiation has been associated with better outcome.1 Treatment should not be delayed until the result of autoantibody testing is available. Case report A 31-year-old man developed a subacute onset of headache, left-sided numbness, and anterograde amnesia. In the following 2 weeks, he experienced personality changes, anxiety, paranoid thoughts, 7 kg weight loss, and worsening cognitive changes. He said that he felt as though he was trapped in a time loop, meaning that events seemed to be constantly recurring to him. He denied fever, night sweats, viral prodromal symptoms, or recent vaccinations. He was ...

Psychosomatics Volume 61, Issue 1, January-February 2020, Pages 64-69 Case Report Delirious Mania as a Neuropsychiatric Presentation in Patients With Anti-N-methyl-D-aspartate Receptor Encephalitis Author links open overlay panel Show more View Abstract © 2019 Academy of Consultation-Liaison Psychiatry. Published by Elsevier Inc. All rights reserved.. ...

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a well-defined autoimmune disorder. Hashimotos encephalopathy (HE) is a still controversial entity, lacking definite diagnostic criteria. We described a 14-year-old-girl presenting with a clinical picture consistent with the diagnosis of anti-NMDAR encephalitis, confirmed by NMDAR antibody testing. Four years earlier, she had presented a similar episode of acute encephalopathy diagnosed as HE. Anti-NMDAR encephalitis and HE share similar clinical features so that the differential diagnosis can be difficult if specific antibodies are not tested. The correct diagnosis of anti-NMDAR encephalitis is crucial to plan the appropriate management and follow-up, namely in term of oncological screening, since it can be paraneoplastic in origin. We suggest to re-evaluate the clinical history of all subjects with previous HE diagnosis in order to evaluate the possible diagnosis of anti-NMDAR encephalitis and plan the appropriate management of these patients

Author and former New York Post writer, Susanah Cahalan, was diagnosed with anti-NMDA receptor encephalitis. This is an acute inflammation of the brain caused by autoimmunity. This diagnosis may also be considered in a differential diagnosis for HE/SREAT and can share symptoms, such as hallucinations and seizures. Ms. Cahalans vivid description of her hallucinations may ring with familiarity to patients diagnosed with HE/SREAT as well.. This video comes form Autoimmune Encephalitis Alliance from their Youtube.com channel, Autoimmune Encephalitis Alliance.. ...

Objective. Our aim was to identify patients with probable anti-NMDA receptor encephalitis among historical medical cases.. Method. A case report published in leading Hungarian-, German- and Italian-language medical journals in the early 1840s was revisited.. Results. In 1830, an 18-year-old, healthy woman suffered epileptic seizures, followed by a 6-day-long state characterized by catalepsy, unresponsiveness, motionless, and light breathing. Her symptoms regularly returned in the following 1.5 years. Meanwhile, a progressively growing huge abdominal tumor appeared. One day, she suddenly started vomiting a large amount of foul-smelling pus mixed with blood, accompanied by bone fragments. Pus mixed blood with some membranous substance was also evacuated through the anus and vagina. After this event, she completely recovered; 1.5 years later, she married and later gave birth to 3 healthy children. The patient remained healthy during the 11-year follow-up.. Conclusions. We suggest that in the ...

By Teresa Conrick In Part 1 of our examination of Anti-NMDA Receptor Encephalitis, I presented a late-onset case of autism. Similar cases have been shown to be caused by antibodies against NR1-NR2 heteromers of the NMDA receptor. There was really...

PubMed journal article: A Case of Severe Anti-N-Methyl D-Aspartate (Anti-NMDA) Receptor Encephalitis with Refractory Autonomic Instability and Elevated Intracranial Pressure. Download Prime PubMed App to iPhone, iPad, or Android

Investigators at Universities of Barcelona, Pennsylvania, Oviedo, and Valencia, and the Spanish NMDAR Encephalitis Work Group report the clinical features of 20 pediatric patients with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis seen in a single center in Spain in the last 4 years. Median patient age was 13 years (range, 8 months-18 years); 70% were female. Initial symptoms were neurologic (dyskinesias or seizures) in 12 (60%) and psychiatric in 40%. By one month after disease onset, all had involuntary movements and changes in behavior and speech. All patients received steroids, IV immunoglobulin or plasma exchange, and 7 rituximab or cyclophosphamide. At a median follow-up of 17.5 months, 85% had substantially recovered, 10% had moderate or severe deficits, and 1 had died. Three patients had previous episodes compatible with anti-NMDAR encephalitis, and 2 had additional relapses. Ovarian teratoma was identified in 2 patients (10%), 1 at disease onset and the other one-year later. A ...

Background: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a recently described life-threatening autoimmune disorder associated with a characteristic multi-stage neuropsychiatric syndrome. Although it is known that the majority of patients experience neuropsychological disturbance post-treatment, some aspects of the cognitive profile remain unclear. Methods: This study sought to investigate patterns of cognitive functioning in a sample of anti-NMDAR encephalitis patients. Seven (6F:1M; mean age, 26.4 years; range, 16-37 years) treated patients completed a comprehensive set of neurocognitive and social functioning measures. Performance was analyzed using normative data (where available), and comparison with matched controls (10F:4M; mean age, 25.8 years; range, 16-38 years). Results: Individual cognitive profiles ranged from within normal limits to extensive dysfunction. Relative to controls, the patient groups performance was affected in the domains of verbal/ visual memory, working ...

When I started working as a Clinical Microbiologist in 2007, anti-NMDA receptor encephalitis had not yet been discovered. The diagnostic test, looking for anti-NMDA receptor antibodies, only appeared commercially around 2010.. Now it is the latest fashionable test to perform…. I am getting old.. The whole area of auto-immune encephalitis has progressed rapidly in the last 10 years, and this potentially treatable cause of encephalitis (options include steroids, IV immunoglobulins, plasma exchange and immunomodulators) is now thought to be similar in prevalence to some viral encephalitides.. Anti-NMDA receptor encephalitis represents the vast majority (approx. 80%) of all cases of autoimmune encephalitis. It usually presents with a short prodromal period followed by a range of symptoms such as auditory and visual hallucinations, delusions, behavioural change, decreased level of consciousness, seizures, and autonomic dysfunction. A majority of patients (58%) with anti-NMDA receptor encephalitis ...

Anti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis is a severe autoimmune condition, which typically affects young females. The long-term clinical consequences and brain morphology changes after anti-NMDAR encephalitis are not well known. We present clinical and neuroimaging follow-up data on a 25-year female patient with typically presenting anti-NMDAR encephalitis. Longitudinal analyses of brain morphology were done using 3 T structural magnetic resonance imaging (sMRI) and Freesurfer analysis at the time of diagnosis and after symptomatic remission. The presented case attained good functional recovery after standard immunoglobulin-corticosteroid treatment but elevated serum NMDAR antibody levels persisted. The patient had no symptomatic relapses during a 3-year clinical follow-up. In the baseline brain sMRI scan there were no marked volume changes. However, a follow-up sMRI after 9 months indicated clear volume reductions in frontal cortical regions compared to matched controls with identical

Encephalitis with antibodies against N-methyl D-aspartate receptor (NMDAR) is recognized as a group of antibody-mediated neuropsychiatric syndromes, which occurs with and without a tumor association. Neoplasm may contribute to the pathogenesis of Anti-NMDAR encephalitis in tumor-positive patients. However, the underlying causes in tumor-negative patients are largely unknown. This is the first report, of which we are aware, of two cases of anti-NMDAR encephalitis after the resection of melanocytic nevus. We describe 2 female patients in their 20s confirmed with anti-NMDAR encephalitis. They shared two points in common: About several weeks (2 weeks and 5 weeks respectively) before the initial symptom, both of them underwent a resection of melanocytic nevi; the screening tests for an ovarian teratoma and other tumors were all negative. A 25 year-old woman presented with seizure, psychiatric symptoms and behavioral change for 2 weeks. Electroencephalogram indicated electrographic seizures. Anti-NMDAR

We determined that this patient had definite paraneoplastic encephalitis, with predominant involvement of the limbic system. Accordingly, she developed a gradual loss of short-term memory, seizures, psychiatric symptoms, MRI abnormalities in the limbic system and positive titres for antineuronal antibodies6-8.. Central nervous system tissue in the teratoma might be a trigger of the immune reaction. The majority of teratomas are mature cystic ones. Immature teratomas (constituting 1% of all teratomas) were present in 29% of anti-NMDAR related cases9. Bilateral teratomas were present in 14% of cases, comparable to 12% described in general.. Development of paraneoplastic syndromes in the setting of gynecologic malignancy is rare. When they occur, the neurologic symptoms most often precede and usually lead to the diagnosis of malignancy. Early diagnosis and aggressive treatment of the underlying tumor may stabilize or improve the neurologic abnormalities. Paraneoplastic neurological syndromes (PLE) ...

In their JNNP paper, Shu et al investigated whether anti-NMDAR encephalitis was associated with any HLA alleles, utilising samples from 61 patients with anti-NMDAR encephalitis and 571 healthy controls from the Chinese Han population.4 In this study, six HLA loci (HLA-A, HLA-B, HLA-C, HLA-DRB1, HLA -DQA1 and HLA -DQB1) were typed using a PCR sequence-based typing method, while the German study mentioned above imputed HLA alleles from single nucleotide polymorphisms (SNPs) genotyped on a genome-wide SNP chip array.2 Shu et al demonstrated that the HLA-DRB1*16:02 allele was associated with disease susceptibility (allele frequency: 14.75% in cases vs 4.82% in controls; OR 3.42 (95% CI 1.82 to 6.17)) and the association did not depend on the positivity of tumours. Furthermore, the patients with anti-NMDAR encephalitis with HLA-DRB1*16:02 tended to respond less efficiently to treatment, suggesting that certain genetic backgrounds may contribute to distinct disease development.. Although replication ...

Tokai J Exp Clin Med. 2012 Sep 20;37(3):89-93. Aoki H, Morita S, Miura N, Tsuji T, Ohnuki Y, Nakagawa Y, Yamamoto I, Takahashi H, Inokuchi S. Source Department of Emergency and Critical Care Medicine, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa 259-1193, Japan. [email protected]. Abstract A previously healthy 21-year-old woman, transported to our…

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One article on anti-NMDA receptor encephalitis noted that the N-methyl-D-aspartate (AKA NMDA) receptor is, an important protein found on the surface of many nerve cells that allows them to receive signals from other nerve cells. The receptor is found throughout the brain and is involved in the formation of new memories as well as in perception and judgement. In anti-NMDA receptor encephalitis a persons immune system for some reason begins creating antibodies that cap the ends of some neurons, preventing nerve signals from crossing the synapses that normally allow the transmission of signals from neuron to neuron. When that happens, the symptoms of schizophrenia or bipolar disorder can appear ...

In this study, anti-NMDARE cases had a higher prevalence of HSV-1 IgG antibodies than age-matched controls in two separate cohorts. Antibodies to unrelated viruses (CMV and EBV-VCA) were not elevated in anti-NMDARE participants, suggesting that this finding is selective for HSV-1. HSV-1 IgG seroprevalence is greater than 60% by mid-adulthood.6 Therefore, we evaluated the association between HSV-1 infection and anti-NMDARE in children, in which reported HSV-1 IgG seroprevalence is only 26% at age 7 years.7 The rate of positive HSV-1 IgG antibodies detected in our participants with anti-NMDARE is elevated compared with this population statistic and the age-matched control group described here. In this retrospective study, with limited demographic information available, we were unable to control for race or socioeconomic status. However, HSV-1 seropositivity in anti-NMDARE participants was greater than that reported for any demographic group in this age range.7 Prospective studies may better ...

Researchers at Johns Hopkins University published a retrospective study in which they examined the PET scans of patients who had been definitively diagnosed with autoimmune encephalitis over a ten-year period. They compared patients with anti-NMDA receptor encephalitis with other subgroups. They found those with anti-NMDAr AE consistently showed abnormalities in the form of hypometabolism (lower metabolism) in a part of the brain called the occipital lobe. The authors recalled that formerly some of the first published cases of anti-NMDAr AE included reports of PET scan abnormalities that included occipital hypometabolism, along with other notable observations.. However they mentioned there has been some uncertainty of the specificity and practicality of using PET in autoimmune encephalitis, as there can be wide variations found in patients in the same subgroup (including anti-NMDA). In addition, all hospitals do not utilize PET scans in the diagnostic workup for AE. Despite these challenges, ...

Went to a fabulous lecture by Dr. Souhel Najjar on autoimmune encephalitis this morning. As a reminder, bad relationships (including with ones self-image etc.) can also cause/contribute to inflammatory burden. Below is a well-informed and written piece on Anti-NMDA-receptor encephalitis (one of many), courtesy of Wikipedia. Last edited 11 days ago by an anonymous user Anti-NMDA receptor encephalitis Watch this page Anti-NMDA (N-methyl D-aspartate) receptor antibody encephalitis, also termed NMDA receptor antibody encephalitis, is an acute form of encephalitis which is potentially lethal but has high probability for … Continue reading →. ...

This page includes the following topics and synonyms: NMDA Encephalitis, Anti-NMDA Receptor Encephalitis, NMDA Receptor Antibody Encephalitis, NMDARE.

The book narrates Cahalans issues with anti-NMDA receptor encephalitis and the process by which she was diagnosed with this form of encephalitis. She wakes up in a hospital with no memory of the events of the previous month, during which time she would have violent episodes and delusions. Her eventual diagnosis is made more difficult by various physicians misdiagnosing her with several theories such as partying too much and schizoaffective disorder.[2] Eventually several physicians, including Dr. Souhel Najjar, began to suspect that Cahalan was suffering from an autoimmune disease.[2] Najjar diagnosed Cahalan using a test that involved her drawing a clock, a test normally given to people suspected of having dementia or Alzheimers disease.[3] Rather than drawing the clock face normally, the disease caused Cahalan to draw all of the numbers 1 through 12 on the right face of the clock, because the right side of her brain, which regulates the left side of the body, was inflamed. Najjar used this ...

We are pleased to inform you that Neuroimmunology at Queen Elizabeth University Hospital Glasgow is now able to offer testing for Anti- glutamate receptor (Type NMDA) antibodies and Anti- voltage gated potassium channel associated proteins (LGI1 and CASPR2).. •Anti- glutamate receptor (Type NMDA) antibodies Anti-NMDA receptor encephalitis manifests along a spectrum of psychosis, altered behaviour, movement disorder, seizures, autonomic dysfunction and decreased consciousness. In younger patients, particularly female, it is associated with an underlying teratoma. Early identification and treatment with immunotherapy leads to better outcomes (Pubmed ID 23290630). It is less common in older patients (over 45 years old) and they display a less severe phenotype and have poorer outcomes (Pubmed ID23946310).. Antibodies against the NR1 subunit of the NMDA receptor are identified in our laboratory via indirect immunofluorescence of cell lines transfected with cDNA coding this protein. This test has ...

A mysterious onset of schizophrenia-like symptoms in patients turned out to be cases of anti-NMDA receptor encephalitis - an autoimmune disorder where antibodies storm the brain.

PGY3 Andrea Cedeno presented a case of a young male with no significant PMH who came in with a few weeks of headache, confusion and change in behavior. After an extensive work-up he was found to have anti-NMDA receptor encephalitis. When evaluating for this disorder, infectious causes (patient had an LP which showed a very…

Steiner (9) tested serum from 459 sufferers with either schizophrenia or other psychiatric disorders for the presence of NMDAR autoimmunity. They found two individuals with GluN1 IgG antibodies, but both experienced classic anti-NMDAR encephalitis with neurologic features, and their conditions had been misdiagnosed. Additional immunoglobulin subtypes were recognized in 10% of individuals with schizophrenia in the initial statement (IgA or IgM antibodies, or both, reacting with GluN1/GluN2 NMDAR subunits). However, subsequent work found that the rate of recurrence of IgA and IgM antibodies realizing NMDARs is similar in control and healthy individuals (10), again casting doubt over the scientific Afatinib relevance of the NMDAR antibody subtypesa bottom line consistent with function by Hammer (5), also displaying ~10% serum positivity of IgM and IgA NMDAR antibodies in sufferers and control topics. Pathmanandavel (1) tested for Afatinib the current presence of NMDAR autoantibodies within a people ...

Every disease needs a patient advocate, and NMDA receptor encephalitis has found its spokesperson in Susannah Cahalan. For several years prior to and during the onset of her illness, Cahalan worked as a journalist at the New York Post, and she tackles the book with the intensity, readability, and bluntness expected of a Post journalist. Here, she finds the balance between being biographical and educational without becoming laden with facts or burdened by irrelevant detail - it is an engaging read.. In Brain on Fire, Cahalan describes her battle with NMDA receptor encephalitis, a condition first well described by neurologist Josep Dalmau, MD, PhD, and colleagues in 2007. The condition, which is is still not widely and commonly recognized, even among physicians, is an acute autoimmune encephalitis caused by immune-mediated attack against NR1- and NR2-subunits of the glutamate NMDA receptor. NMDA encephalitis is often associated with ovarian teratoma when diagnosed in young women, though it can ...

Tijdschr Psychiatr. 2012;54(3):279-83. [Article in Dutch] Waas JA, Storm AH. Source Accare, locatie de Ruyterstee, Fazantenlaan 1, 9422 EZ Smilde. [email protected] Abstract We describe the case of a 17-year-old girl with anti-NMDA-receptor encephalitis. She had the characteristic psychiatric symptoms such as hallucinations, delirious and bizarre behaviour, and catatonic symptoms. She later also displayed neurological symptoms…

Autoimmune synaptic encephalitis can occur as paraneoplastic neurological syndromes, which are dysfunctions of the nervous system in cancer patients. One such rare but treatable form is associated wit...

First-line immunotherapy was often insufficient to control the disease; in the group of patients in the cited study, the addition of second-line immunotherapy was associated with a better outcome compared with those not receiving second-line immunotherapy.4 Immunotherapy and tumor removal, if applicable, result in substantial neurological improvement in 81% of patients with anti-NMDAR encephalitis after a median follow-up of 24 months. Moreover, second-line immunotherapy with rituximab, cyclophosphamide, or both, improved the outcome of patients who did not respond to first-line treatment and decreased the occurrence of relapses.4 However, 25% of patients suffer from severe neurological deficits or die. Upon recovery, amnesia is evident for the duration of the illness, and there is a risk of relapse of the encephalitic syndrome.6,7 The gradual process of clinical recovery may take a long time (sometimes years) but can, in some patients, even be associated with improvement of frontotemporal brain ...

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PubMed journal article: 18F-FDG PET/CT in Initial Diagnosis and Treatment Response Evaluation of Anti-NMDAr and Anti-GAD Dual Antibody Autoimmune Encephalitis. Download Prime PubMed App to iPhone, iPad, or Android

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Antibody-associated central nervous system (CNS) autoimmune diseases account for an important part in neuroimmunology. Early studies mainly focused on paraneoplastic onconeural antibodies with a target on intracellular antigens (such as Hu, Yo, Ri, etc.). The past decade has witnessed a surge of discovery of novel neural antibodies, along with a series of new CNS disorders mediated by those antibodies, such as autoimmune encephalitis represented by anti-N-methyl-D-aspartate receptor encephalitis, aquaporin 4 antibody-positive neuromyelitis optica spectrum disorder, myelin oligodendrocyte glycoprotein antibody-associated disease, and autoimmune glial fibillary acidic protein astrocytopathy.With a surge of antibody-associated CNS disorders, clinicians are faced with emerging challenges, including (1) the clinical significance and diagnostic value of antibodies; (2) the diagnostic dilemma in circumstances of

John Corboy, Editor of Neurology: Clinical Practice introduces the December 2014 Special Issue dedicated to Case Reports. The Editors then interview authors of three featured Case Reports. David Anderson, Associate Editor for Neurology: Clinical Practice, interviews W. Oliver Tobin about a Case titled NMDA receptor encephalitis causing reversible caudate changes on MRI and PET imaging; John Corboy interviews Kinga Szigeti about a Case titled Capgras-like visual decomposition in Lewy body dementia with therapeutic response to donepezil; and Richard Barbano, Associate Editor for Neurology: Clinical Practice, interviews Jamie Ad Read More John Corboy, Editor of Neurology: Clinical Practice introduces the December 2014 Special Issue dedicated to Case Reports. The Editors then interview authors of three featured Case Reports. David Anderson, Associate Editor for Neurology: Clinical Practice, interviews W. Oliver Tobin about a Case titled NMDA receptor encephalitis causing reversible caudate ...

BACKGROUND: Paraneoplastic neurological syndromes (PNNS) are remote effects of a tumor and mediated by an altered immune reaction. In the last ten years, the spectrum of PNNS has changed profoundly with the discovery of a new category of neurological diseases that are associated with antibodies against surface or synaptic antigens. In contrast to classical PNNS, patients with surface receptor autoimmunity are often highly responsive to immunotherapy. OBJECTIVES: This article provides an update on the most relevant PNNS, focusing on specific syndromes associated with antibodies against classical onconeuronal antigens as well as surface and synaptic proteins ...

Howard Forman (HF): In Brain on Fire, your debut book that has been called by NPR stunningly brave and a gift of a book from one of Americas most courageous young journalists, you write about some harrowing experiences that occurred during 2009, when you were 24 years old. Can you describe some of the symptoms that you experienced during that time?. Susannah Cahalan (SC): The earliest symptoms were subtle. At first, I just felt off. I was tired and emotional. I became fixated on certain things: I believed I had bedbugs to the point that I urged an exterminator to debug my apartment even when he insisted I didnt have an infestation. I thought my boyfriend was cheating on me and went in search of nonexistent clues of his philandering.. Then, I felt numbness on the left side of my body, which my neurologist ascribed to mono. But I didnt link my odd feelings with this physical experience.. Later, my behavior became increasingly irrational. My moods fluctuated from extreme happiness to ...

A form of encephalitis associated with anti-N-methyl-d-aspartate receptor (anti-NMDA-R) antibodies has recently been described.1 Reported patients are mainly young women, presenting with severe encephalitis and additional distinctive neurological features. Around 60% have an ovarian teratoma.1 The severe course of the disease does not rule out favourable prognosis. Immunotherapy is advocated1-3 and appears to be associated with improved outcome.. We present a patient with anti-NMDA-R encephalitis and serial [18F]-fluorodeoxyglucose-positron emission tomography (FDG-PET) examinations showing markedly increased activity in the basal ganglia as compared with that in the cortex when extrapyramidal features were prominent, which normalised after improvement of this movement disorder. ...

Over the last years it has become evident that many neurological diseases of the central nervous system (CNS) are induced by a specific adaptive immune response directed against molecules expressed on CNS-resident cells. Well-recognized examples are anti-N-Methyl-D-Aspartate Receptor (NMDAR) encephalitis which is characterized by the presence of antibodies against neuron-expressed NMDAR, or neuromyelitis optica (NMO), induced by antibodies to astrocyte-expressed aquaporin-4. Many more examples exist, and antibodies, and T or/and B cells have increasingly been associated with CNS disease. Often the symptoms of these diseases have not been typically reported to have an immune aetiology. Beside classical neurological symptoms like ataxia, vision disturbance, and motor or sensory symptoms, these can include cognitive disturbances, behavioral abnormalities, or/and epileptic seizures. Although much has been learned regarding the pathophysiology of prototypic examples of these disorders, there are ...

OBJECTIVE: We tested whether antibody screening samples of patients with suspected autoimmune encephalitis with additional research assays would improve the detection of autoimmune encephalitis compared with standard clinical testing alone. METHODS: We examined 731 samples (333 CSF, 182 sera, and 108 pairs) from a cohort of 623 patients who were tested for CNS autoantibodies by the University of Pennsylvania clinical laboratory over a 24-month period with cell-based assays (CBAs) on commercially obtained slides of fixed cells for antibodies to NMDA receptor (NMDAR), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), γ-aminobutyric acid-B receptor (GABABR), leucine-rich glioma-inactivated 1 (LGI1), contactin-associated protein-like 2 (Caspr2), and glutamic acid decarboxylase (GAD65 ...

Hi experts, I searched this forum re. getting insurance approval for IVIG and asked on an older post that was for AE; our MDs say thats out b/c its not anti-NMDA AE, so I thought Id try to be more specific in my question here. Im preparing my insurance appeal in case UHC denies coverage for out-patient 2-days of IVIG, although theyve expedited the review process. Questions: 1. How did those of you who had to appeal your insurances first denial of IVIG (for hypogammaglobulinemia) approach it, eg. did you list PANS/PANDAS as a dx ?we were told by both our PANS NP/RN and ID MD t ...

1. Weinmann-Menke J, Holtz S, Sollinger D, Dörken M, Boedecker S, Schamberger S, Pfister F, Amann K, Lutz J. Treatment of Membranous Nephropathy in patients with Thrombospondin Type 1 -Domain-Containing 7A (THSD7A) Antibodies using Immunoadsorption. AJKD 2019 in press.. 2. Schwarting A, Möckel T, Lütgendorf F, Triantafyllias K, Grella S, Boedecker S, Weinmann A, Lüssi F, Meineck M, Sommer C, Schermuly I, Fellgiebel A, Weinmann-Menke J. Fatigue in SLE - diagnostic and pathogenic impact of anti-N-methyl-D-aspartate receptor (NMDAR) autoantibodies. Ann Rheum Dis. 2019 in press. 3. Triantafyllias K, de Blasi M, Lütgendorf F, Cavagna L, Stortz M, Weinmann-Menke J, Konstantinides S, Galle PR, Schwarting A. High cardiovascular risk in mixed connective tissue disease: evaluation of macrovascular involvement and its predictors by aortic pulse wave velocity. Clin Exp Rheumatol. 2019 April 2. 4. *Wada Y, *Gonzalez-Sanchez HM, *Weinmann-Menke J, Iwata Y, Ajay AK, Meineck M, Kelley VR. IL-34-Dependent ...