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Autoimmune encephalitis

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https://www.readbyqxmd.com/read/28424014/semi-quantitative-analysis-of-cerebral-fdg-pet-reveals-striatal-hypermetabolism-and-normal-cortical-metabolism-in-a-case-of-vgkcc-limbic-encephalitis
#1
Patrick Moloney, Ruth Boylan, Marwa Elamin, Sean O'Riordan, Ronan Killeen, Christopher McGuigan
In the context of delayed autoimmune encephalitis antibody results, functional imaging can support the diagnosis of limbic encephalitis associated with anti-voltage-gated potassium channel complex (VGKCC) antibodies. Here we present a typical case of VGKCC encephalitis in a 69-year-old woman whose symptoms responded to plasmapheresis. A cerebral 18F-fluoro-2-deoxy-d-glucose positron emission tomography (FDG-PET) scan performed prior to commencing treatment revealed striatal hypermetabolism assessed qualitatively and semi-quantitatively, with normal uptake in the cortex and cerebellum when analysed semi-quantitatively...
April 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/28423529/pif-promotes-brain-re-myelination-locally-while-regulating-systemic-inflammation-clinically-relevant-multiple-sclerosis-m-smegmatis-model
#2
Giuseppe Migliara, Martin Mueller, Alessia Piermattei, Chaya Brodie, Michael J Paidas, Eytan R Barnea, Francesco Ria
Neurologic disease diagnosis and treatment is challenging. Multiple Sclerosis (MS) is a demyelinating autoimmune disease with few clinical forms and uncertain etiology. Current studies suggest that it is likely caused by infection(s) triggering a systemic immune response resulting in antigen/non-antigen-related autoimmune response in central nervous system (CNS). New therapeutic approaches are needed. Secreted by viable embryos, PreImplantation Factor (PIF) possesses a local and systemic immunity regulatory role...
March 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28418206/anti-nmda-receptor-encephalitis-during-pregnancy-a-case-report-and-literature-review
#3
Xue Xiao, Shunping Gui, Peng Bai, Yi Bai, Dan Shan, Yayi Hu, Tri M Bui-Nguyen, Rong Zhou
Anti-N-methyl-d-aspartate receptor (anti-NMDA-R) encephalitis is an autoimmune disorder that was first described by Dr Vitaliani in 2005. In 2007, Dalmau et al. found anti-NMDA-R antibody expressed both in the hippocampus and prefrontal nerve cell membrane, finally proposing the diagnosis of autoimmune anti-NMDA-R encephalitis. Most of the patients are female (91%), with ages ranging from 4 to 76 years. The average age is 23 years, a birth peak age, although anti-NMDA-R encephalitis is rare during pregnancy...
April 2017: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/28417344/biotherapy-in-inflammatory-diseases-of-the-cns-current-knowledge-and-applications
#4
REVIEW
Nicolas Collongues, Laure Michel, Jérôme de Seze
Biotherapy represents an innovative therapeutic approach that includes immunotherapy (vaccines, apheresis, and antibodies); gene therapy; and stem cell transplants. Their development helps to cross the bridge from bench to bedside and brings new hope of a cure for severe diseases in different fields of medicine. In neurology, a growing range of applications is being developed for these medications. Valuable results are now available in the field of autoimmunity, neuro-oncology, paraneoplastic manifestations, and neurodegenerative disorders...
May 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28394968/-autoimmune-encephalitis-induced-by-antibodies-against-gaba-a-receptor
#5
Pablo González R, Lorena Hudson A, Esteban Basáez M, Marcelo Miranda C
Among autoimmune encephalitides, a prevalent group are those associated with antibodies against the N-Methyl-D-aspartate receptor, which present with behavior abnormalities, psychosis, seizures and abnormal movements. A new variant, mediated by antibodies against the GABA-A receptor, was recen-tly described. We report a 66-years-old female with this form of encephalitis whose main manifestation was the presence of severe seizures leading to status epilepticus. The patient had a good response to immunomodulatory therapy with intravenous methylprednisolone, azathioprine and anticonvulsants...
November 2016: Revista Médica de Chile
https://www.readbyqxmd.com/read/28386263/adaptive-immunity-is-the-key-to-the-understanding-of-autoimmune-and-paraneoplastic-inflammatory-central-nervous-system-disorders
#6
REVIEW
Robert Weissert
There are common aspects and mechanisms between different types of autoimmune diseases such as multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSDs), and autoimmune encephalitis (AE) as well as paraneoplastic inflammatory disorders of the central nervous system. To our present knowledge, depending on the disease, T and B cells as well as antibodies contribute to various aspects of the pathogenesis. Possibly the events leading to the breaking of tolerance between the different diseases are of great similarity and so far, only partially understood...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28374703/-autoimmune-anti-nmda-r-encephalitis
#7
E E Vasenina, O S Levin, O A Gan'kina, A Sh Chimagomedova, D I Levikov
Anti-NMDA-R encephalitis is a relatively frequent form of autoimmune encephalitis. Initial clinical features of anti-NMDA-R encephalitis resemble those of schizophrenia exacerbation which resulted in hospitalization of patients to mental care facilities. Taking into account high lethality and potential curability, detection of this condition is an important clinical problem. The authors describe a case report of encephalitis with NMDA-R antibodies. This report is the first in the domestic literature. The difficulties of timely diagnosis, diagnostic criteria and treatment algorithm are presented...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28365191/-thymoma-and-autoimmune-diseases
#8
Y Jamilloux, H Frih, C Bernard, C Broussolle, P Petiot, N Girard, P Sève
The association between thymoma and autoimmunity is well known. Besides myasthenia gravis, which is found in 15 to 20% of patients with thymoma, other autoimmune diseases have been reported: erythroblastopenia, systemic lupus erythematosus, inflammatory myopathies, thyroid disorders, Isaac's syndrome or Good's syndrome. More anecdotally, Morvan's syndrome, limbic encephalitis, other autoimmune cytopenias, autoimmune hepatitis, and bullous skin diseases (pemphigus, lichen) have been reported. Autoimmune diseases occur most often before thymectomy, but they can be discovered at the time of surgery or later...
March 29, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28363946/emergence-of-new-onset-psychotic-disorder-following-recovery-from-lgi1-antibody-associated-limbic-encephalitis
#9
Thomas A Pollak, Nick Moran
Neuronal autoantibodies targeting cell surface antigens have been described in association with autoimmune encephalitides which frequently feature psychosis and other psychiatric disturbances alongside neurological signs and symptoms. Little has been written however about the long-term psychiatric status of individuals following recovery from the acute phase of autoimmune encephalitis, despite case series and anecdotal evidence suggesting this may be a cause of considerable disability. Here, we describe a man aged 58 years with no psychiatric history who developed a severe and acute psychotic disorder following resolution of a protracted course of limbic encephalitis associated with antibodies to leucine-rich glioma inactivated 1 protein...
March 31, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28360986/confusion-faciobrachial-dystonic-seizures-and-critical-hyponatremia-in-a-patient-with-voltage-gated-potassium-channel-encephalitis
#10
Julian Yaxley
Autoimmune limbic encephalitis is a rare cause of encephalitic disease. It is associated with various target antigens and is difficult to diagnose, and experience with its treatment is limited. This case report describes a 69-year-old man, who presented with life-threatening hyponatremia and confusion, following several months of gradually worsening faciobrachial dystonic seizures. Faciobrachial dystonic seizures are a well-described feature classically observed in voltage-gated potassium channel autoimmune encephalitis...
March 2017: Korean Journal of Family Medicine
https://www.readbyqxmd.com/read/28351601/-organic-troubles-with-psychiatric-symptoms-what-is-the-appropriate-childhood-and-adolescence-psychiatric-care-reflections-on-disimmune-encephalitis-cases
#11
R Ozelle, A Doudard, A-L Bodin, S Gueden, P Duverger, E Riquin
Organic mental disorders are different and further revealed by increasingly advanced research. They are nevertheless misunderstood, without consensus, and raise clinical, diagnostic, and therapeutic questions. These disorders require effective collaboration between practitioners such as pediatricians and child psychiatrists. The subject should not disappear behind the complexity related to the clinical expression of these symptoms. Based on three cases of autoimmune encephalitis, we offer a reflection on the management and assessment of these diseases by a multidisciplinary team with the intention of providing optimal management...
May 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28348963/unilateral-predominance-of-abnormal-movements-a-characteristic-feature-of-the-pediatric-anti-nmda-receptor-encephalitis
#12
Vanessa Benjumea-Cuartas, Monika Eisermann, Hina Simonnet, Marie Hully, Rima Nabbout, Isabelle Desguerre, Anna Kaminska
Anti-NMDA receptor encephalitis is a treatable autoimmune disease characterized by cognitive, motor and psychiatric features that primarily affects young adults and children. We present a case of a 7-year-old boy with asymmetrical (mainly right hemibody) and abnormal polymorphic movements without concomitant scalpictal EEG changes but had background slowing predominating over the left hemisphere. This report illustrates previous descriptions of asymmetric presentation of abnormal movements in pediatric anti-NMDA receptor encephalitis and emphasizes the importance of video-EEG interpreted within the overall clinical context, to differentiate epileptic from non-epileptic abnormal movements in patients with autoimmune encephalitis...
2017: Epilepsy & Behavior Case Reports
https://www.readbyqxmd.com/read/28348631/optic-neuropathy-as-the-initial-presenting-sign-of-n-methyl-d-aspartate-nmda-encephalitis
#13
Mark Mugavin, Brett H Mueller, Masoom Desai, Karl C Golnik
A 52-year-old woman presented with painless vision loss for 3 months. She was in custody for allegedly robbing a bank and had recently been diagnosed with paranoid schizophrenia. She had 20/100 VA OD, a 2+RAPD, and optic atrophy. Extensive diagnostic workup including MRI, Fluorescein Angiography, Infectious Disease Panel, lumbar puncture, and leptomeningeal biopsy were unrevealing. Vision in her right eye declined to NLP and her left eye declined to 20/200 VA. Anti N-methyl-D-aspartate (NMDA) Autoimmune Encephalitis was diagnosed based on CSF serology and clinical suspicion...
April 2017: Neuro-ophthalmology
https://www.readbyqxmd.com/read/28348532/ethical-implications-of-the-mild-encephalitis-hypothesis-of-schizophrenia
#14
Rita Riedmüller, Sabine Müller
Schizophrenia is a serious mental disease with a high mortality rate and severe social consequences. Due to insufficient knowledge about its etiopathogenesis, curative treatments are not available. One of the most promising new research concepts is the mild encephalitis hypothesis of schizophrenia, developed mainly by Karl Bechter and Norbert Müller. According to this hypothesis, a significant subgroup of schizophrenia patients suffer from a mild, but chronic, form of encephalitis with markedly different etiologies ranging from viral infections, traumas to autoimmune diseases...
2017: Frontiers in Psychiatry
https://www.readbyqxmd.com/read/28345736/-kleine-levin-syndrome-differential-diagnosis-in-recurrent-encephalitic-syndromes-in-adolescence
#15
A Duat-Rodriguez, I Martinez-Albadalejo, I Perez-Sebastian, V Cantarin-Extremera, A Hedrera-Fernandez, J J Garcia-Penas
INTRODUCTION: The Kleine-Levin syndrome is a rare disease of unknown origin characterized by recurrent and self-limited episodes of hypersomnia that are also accompanied by a cognitive and behavioral dysfunction. Patients present normal sleeping and behavior patterns between episodes. CASE REPORTS: We present three patients who are 14 years old: two boys and one girl. They started having the episodes after a predisposing factor (vaccine, influenza B and menstruation)...
April 1, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28314470/infectious-encephalitis-management-without-etiological-diagnosis-48hours-after-onset
#16
REVIEW
P Fillatre, Y Crabol, P Morand, L Piroth, J Honnorat, J P Stahl, M Lecuit
INTRODUCTION: The etiological diagnosis of infectious encephalitis is often not established 48hours after onset. We aimed to review existing literature data before providing management guidelines. METHOD: We performed a literature search on PubMed using filters such as "since 01/01/2000", "human", "adults", "English or French", and "clinical trial/review/guidelines". We also used the Mesh search terms "encephalitis/therapy" and "encephalitis/diagnosis". RESULTS: With Mesh search terms "encephalitis/therapy" and "encephalitis/diagnosis", we retrieved 223 and 258 articles, respectively...
March 15, 2017: Médecine et Maladies Infectieuses
https://www.readbyqxmd.com/read/28306571/cns-syndromes-associated-with-antibodies-against-metabotropic-receptors
#17
Eric Lancaster
PURPOSE OF REVIEW: Autoantibodies to Central nervous system (CNS) metabotropic receptors are associated with a growing family of autoimmune brain diseases, including encephalitis, basal ganglia encephalitis, Ophelia syndrome, and cerebellitis. The purpose of this review is to summarize the state of knowledge regarding the target receptors, the neurological autoimmune disorders, and the pathogenic mechanisms. RECENT FINDINGS: Antibodies to the γ-aminobutyric acid B receptor are associate with limbic encephalitis and severe seizures, often with small cell lung cancers...
March 16, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28302135/myeloid-c-ebp%C3%AE-deficiency-reshapes-microglial-gene-expression-and-is-protective-in-experimental-autoimmune-encephalomyelitis
#18
Marta Pulido-Salgado, Jose M Vidal-Taboada, Gerardo Garcia Diaz-Barriga, Joan Serratosa, Tony Valente, Paola Castillo, Jonathan Matalonga, Marco Straccia, Josep M Canals, Annabel Valledor, Carme Solà, Josep Saura
BACKGROUND: CCAAT/enhancer binding protein β (C/EBPβ) is a transcription factor that regulates the expression of important pro-inflammatory genes in microglia. Mice deficient for C/EBPβ show protection against excitotoxic and ischemic CNS damage, but the involvement in this neuroprotective effect of the various C/EBPβ-expressing cell types is not solved. Since C/EBPβ-deficient microglia show attenuated neurotoxicity in culture, we hypothesized that specific C/EBPβ deficiency in microglia could be neuroprotective in vivo...
March 16, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28298911/autoantibodies-against-the-n-methyl-d-aspartate-receptor-subunit-nr1-untangling-apparent-inconsistencies-for-clinical-practice
#19
REVIEW
Hannelore Ehrenreich
This viewpoint review provides an integrative picture of seemingly contradictory work published on N-methyl-d-aspartate receptor 1 (NMDAR1) autoantibodies (AB). Based on the present state of knowledge, it gives recommendations for the clinical decision process regarding immunosuppressive treatment. Brain antigen-directed AB in general and NMDAR1-AB in particular belong to a preexisting autoimmune repertoire of mammals including humans. Specific autoimmune reactive B cells may get repeatedly (perhaps transiently) boosted by various potential stimulants (e...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28284337/autoimmune-limbic-encephalitis-with-anti-contactin-associated-protein-like-2-antibody-secondary-to-pembrolizumab-therapy
#20
Michael P Brown, Pravin Hissaria, Amy Hc Hsieh, Christopher Kneebone, Wilson Vallat
Immune checkpoint inhibitors such as Pembrolizumab are used to restore antitumour immune response. It is important to be vigilant of immune mediated adverse events related to such therapy. We report a case of autoimmune limbic encephalitis with Contactin-Associated Protein-like 2 (CASPR2) antibody secondary to Pembrolizumab therapy for metastatic melanoma.
April 15, 2017: Journal of Neuroimmunology
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