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Autoimmune encephalitis

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https://www.readbyqxmd.com/read/28810906/atypical-presentation-of-anti-n-methyl-d-aspartate-receptor-encephalitis-two-case-reports
#1
Maria Cristina Maggio, Greta Mastrangelo, Aldo Skabar, Alessandro Ventura, Marco Carrozzi, Giuseppe Santangelo, Francesca Vanadia, Giovanni Corsello, Rolando Cimaz
BACKGROUND: Anti-N-methyl-D-aspartate receptor encephalitis is a rare autoimmune disease characterized by severe neurological and psychiatric symptoms and a difficult diagnosis. The disease is often secondary to a neoplastic lesion, seldom diagnosed years later. Psychiatric symptoms are prevalent in adults; neurologic symptoms are more evident in children, who typically present primarily with neurological symptoms. To the best of our knowledge, the association with juvenile idiopathic arthritis has not been described...
August 16, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28804482/risk-factors-for-intensive-care-unit-admission-in-patients-with-autoimmune-encephalitis
#2
Gayane Harutyunyan, Larissa Hauer, Martin W Dünser, Tobias Moser, Slaven Pikija, Markus Leitinger, Helmut F Novak, Wolfgang Aichhorn, Eugen Trinka, Johann Sellner
BACKGROUND: Prevention and early recognition of critical illness in patients with autoimmune encephalitis (AE) is essential to achieve better outcome. AIM OF THE STUDY: To evaluate risk factors for intensive care unit (ICU) admission and its prognostic impact in patients with AE. PATIENTS AND METHODS: A reclassification of patients hospitalized between 2011 and 2016 revealed 17 "definite" and 15 "probable" AE cases. Thirteen patients (41%) developed critical illness and required ICU admission...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28794080/the-immune-system-in-pediatric-seizures-and-epilepsies
#3
REVIEW
Christian M Korff, Russell C Dale
The relation between the immune system and epilepsy has been studied for a long time. Immune activation may precede or follow the appearance of seizures. Depending on the situation, the innate and acquired immunity may be involved to various degrees. The intense, ongoing research has opened encouraging management and therapeutic perspectives for a significant number of patients suffering from seizures. These include the use of various drugs and less conventional approaches with anti-inflammatory or immunomodulatory properties...
August 9, 2017: Pediatrics
https://www.readbyqxmd.com/read/28789769/-autoimmune-encephalitis-presenting-with-drug-resistant-status-epilepticus
#4
Mette Nissen, Morten Blaabjerg
Autoimmune encephalitis is characterized by formation of antibodies against cell surface proteins. Antibodies against the gamma-aminobuturic acidB (GABAB) receptor lead to limbic encephalitis, drug-resistant seizures, confusion, cognitive impairment and changed behaviour. Some patients present with status epilepticus. GABAB encephalitis is associated with small-cell lung cancer or neuroendocrine lung tumour. This is a case report of a patient having status epilepticus due to GABAB encephalitis. In cases which are presumed to be autoimmune it is important that treatment starts immediately instead of awaiting antibody confirmation...
July 24, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/28778449/heparanase-potential-roles-in-multiple-sclerosis
#5
REVIEW
Benjarat Changyaleket, Zane Deliu, Andreia Z Chignalia, Douglas L Feinstein
Heparanase is a heparan sulfate degrading enzyme that cleaves heparan sulfate (HS) chains present on HS proteoglycans (HSPGs), and has been well characterized for its roles in tumor metastasis and inflammation. However, heparanase is emerging as a contributing factor in the genesis and severity of a variety of neurodegenerative diseases and conditions. This is in part due to the wide variety of HSPGs on which the presence or absence of HS moieties dictates protein function. This includes growth factors, chemokines, cytokines, as well as components of the extracellular matrix (ECM) which in turn regulate leukocyte infiltration into the CNS...
September 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28778447/neuroendocrine-tumour-metastatic-brain-disease-during-immunosuppressive-treatment-for-paraneoplastic-gabab-receptor-antibodies-encephalitis-is-immunosuppression-always-beneficial
#6
Ana André, Ana Félix, Motasem Shamasna, Hipólito Nzwalo, Carlos Basílio
BACKGROUND: Limbic autoimmune encephalitis (LE) should be considered in any patient with acute or subacute neuropsychiatric manifestations, without other common causes of encephalitis. Y-Aminobutyric-acid-B-receptor (anti-GABABR) antibodies are rarely encountered in association with LE. CASE REPORT: A 74-year-old patient presented with a progressive cognitive degradation and generalized tonic-clonic seizures, with positive anti-GABABR. He declined under immunosuppression treatment...
September 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28778439/autoimmune-encephalitis-associated-with-vitiligo
#7
Ren Haitao, Liu Huiqin, Qu Tao, Yang Xunzhe, Shao Xiaoqiu, Li Wei, Zhang Jiewen, Cui Liying, Guan Hongzhi
The autoimmune encephalitis can develop with or without an underlying tumor. For tumor-negative autoimmune encephalitis, the causes are still largely unknown. Here we presented three patients with autoimmune encephalitis accompanied with vitiligo. Among them, two patients suffered from anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis and one patient suffered from anti-IgLON5 encephalopathy. All of them received intravenous immunoglobulin and steroids as immunotherapy. The two patients with anti-LGI1 encephalitis recovered and got a good prognosis...
September 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28777260/persistence-of-parenchymal-and-perivascular-t-cells-in-treatment-refractory-anti-n-methyl-d-aspartate-receptor-encephalitis
#8
Alexander Filatenkov, Timothy E Richardson, Elena Daoud, Sarah F Johnson-Welch, Denise M Ramirez, Jose Torrealba, Benjamin Greenberg, Nancy L Monson, Veena Rajaram
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disease mediated by IgG1 or IgG3 antibodies to the GluN1 subunit of the NMDAR, resulting in downregulation of NMDARs. Early diagnosis, prompt reduction of anti-NMDAR antibodies, and removal of associated ovarian tumors when identified are important drivers of prognosis. Immunohistochemical studies were carried out to evaluate B cell, plasma cell, and T-cell infiltrates in the brain of a 3-year-old patient with anti-NMDAR encephalitis who failed to show improvement after plasma exchange and Rituximab treatment...
August 2, 2017: Neuroreport
https://www.readbyqxmd.com/read/28770118/new-onset-insomnia-in-a-pediatric-patient-a-case-of-anti-nmda-receptor-encephalitis
#9
Tamar N Goldberg, Michael F Cellucci
Anti-NMDAR encephalitis is becoming more widely recognized as a cause of encephalopathy in both adults and children. Certain clinical features such as mood lability, movement disorders, speech dysfunction, seizures, and autonomic instability in a pediatric patient should prompt immediate concern and evaluation for autoimmune encephalitis among providers. We present the case of a pediatric patient with anti-NMDAR encephalitis in which the symptom prompting medical evaluation was insomnia. Insomnia has not previously been emphasized in the literature as a presenting feature of this disease in children and has a broad differential...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28750498/emerging-clinical-issues-and-multivariate-analyses-in-pet-investigations
#10
Javier Arbizu, Alessandro Giuliani, Jaime G Perez-Larraya, Mario Riverol, Cathrine Jonsson, Berta García-García, Maribel Morales, Laura Imaz, Marco Pagani
PET using 18F-2-fluoro-2-deoxy-D-glucose (FDG-PET) has been gradually introduced in the diagnostic clinical criteria of the most prevalent neurodegenerative diseases. Moreover, an increasing amount of literature have shown that the information provided by FDG-PET enhances the sensitivity of standard imaging biomarkers in less frequent disorders in which an early differential diagnosis can be of paramount relevance for patient management and outcome. Therefore emerging uses of FDG-PET may be important in prion diseases, autoimmune encephalitis and amyotrophic lateral sclerosis...
July 27, 2017: Quarterly Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28750305/progressive-hippocampal-sclerosis-after-viral-encephalitis-potential-role-of-nmda-receptor-antibodies
#11
Stoyan Popkirov, Fatme Seval Ismail, Wenke Grönheit, Monika Kapauer, Jörg Wellmer, Christian G Bien
PURPOSE: Survivors of viral encephalitis can develop refractory epilepsy and hippocampal sclerosis. Both the initial infectious insult and the secondary effects of recurrent seizures have been implicated in chronic disease progression. Recently, post-infectious autoimmunity, involved in acute relapses, has also been proposed as a pathomechanism for chronic disease progression. Our case series suggests a potential role of antibodies against the N-methyl-d-aspartate receptor (NMDAR) in chronic inflammatory disease beyond acute manifestations...
July 19, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28741102/the-range-of-neurological-complications-in-chikungunya-fever
#12
T Cerny, M Schwarz, U Schwarz, J Lemant, P Gérardin, E Keller
BACKGROUND: Chikungunya fever is a globally spreading mosquito-borne disease that shows an unexpected neurovirulence. Even though the neurological complications have been a major cause of intensive care unit admission and death, to date, there is no systematic analysis of their spectrum available. OBJECTIVE: To review evidence of neurological manifestations in Chikungunya fever and map their epidemiology, clinical spectrum, pathomechanisms, diagnostics, therapies and outcomes...
May 25, 2017: Neurocritical Care
https://www.readbyqxmd.com/read/28739848/neurosyphilis-mimicking-autoimmune-encephalitis-in-a-52-year-old-man
#13
Adrian Budhram, Michael Silverman, Jorge G Burneo
No abstract text is available yet for this article.
July 24, 2017: CMAJ: Canadian Medical Association Journal, Journal de L'Association Medicale Canadienne
https://www.readbyqxmd.com/read/28725222/neuronal-surface-autoantibodies-in-neuropsychiatric-disorders-are-there-implications-for-depression
#14
REVIEW
Shenghua Zong, Carolin Hoffmann, Marina Mané-Damas, Peter Molenaar, Mario Losen, Pilar Martinez-Martinez
Autoimmune diseases are affecting around 7.6-9.4% of the general population. A number of central nervous system disorders, including encephalitis and severe psychiatric disorders, have been demonstrated to associate with specific neuronal surface autoantibodies (NSAbs). It has become clear that specific autoantibodies targeting neuronal surface antigens and ion channels could cause severe mental disturbances. A number of studies have focused or are currently investigating the presence of autoantibodies in specific mental conditions such as schizophrenia and bipolar disorders...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28720552/autoimmune-limbic-encephalitis-associated-with-type-1-diabetes-mellitus
#15
Onur Akın, Aylin Kılınç Uğurlu, Emine Demet Akbaş, Esra Döğer, Yılmaz Akbaş, Aysun Bideci, Özge Yüce, Kıvılcım Gücüyener, Orhun Çamurdan, Neşe Karabacak, Peyami Cinaz
Glutamic acid decarboxylase (GAD) antibodies can participate in the pathogenesis of both type 1 diabetes mellitus (T1DM) and limbic encephalitis (LE). However, an association between T1DM and LE is very rare. We report a patient who was diagnosed with type 1 diabetes mellitus six months after encountering limbic encephalitis. A 16-year-old boy was admitted with behavioral changes, confusion, and headache. He was diagnosed with LE. Anti-GAD levels were high. He was treated with pulse methylprednisolone. During the steroid treatment, neurological symptoms improved but hyperglycemia occurred...
July 17, 2017: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/28720215/autoimmune-meningitis-and-encephalitis-in-adult-onset-still-disease-case-report
#16
Bożek Milena, Konopko Magdalena, Wierzba-Bobrowicz Teresa, Witkowski Grzegorz, Makowicz Grzegorz, Sienkiewicz-Jarosz Halina
INTRODUCTION: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown cause. Its symptoms usually include persistent fever, fugitive salmon-colored rash, arthritis, sore throat (not specific), but it may also lead to internal organs' involvement, which presents with enlargement of the liver and spleen, swollen lymph nodes, carditis or pleuritis - potentially life-threatening complications. In rare cases, AOSD can cause aseptic meningitis or/and encephalitis...
July 8, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/28716943/visualizing-context-dependent-calcium-signaling-in-encephalitogenic-t-cells-in-vivo-by-two-photon-microscopy
#17
Nikolaos I Kyratsous, Isabel J Bauer, Guokun Zhang, Marija Pesic, Ingo Bartholomäus, Marsilius Mues, Ping Fang, Miriam Wörner, Stephanie Everts, Joachim W Ellwart, Joanna M Watt, Barry V L Potter, Reinhard Hohlfeld, Hartmut Wekerle, Naoto Kawakami
In experimental autoimmune encephalitis (EAE), autoimmune T cells are activated in the periphery before they home to the CNS. On their way, the T cells pass through a series of different cellular milieus where they receive signals that instruct them to invade their target tissues. These signals involve interaction with the surrounding stroma cells, in the presence or absence of autoantigens. To portray the serial signaling events, we studied a T-cell-mediated model of EAE combining in vivo two-photon microscopy with two different activation reporters, the FRET-based calcium biosensor Twitch1 and fluorescent NFAT...
August 1, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28704576/psychosis-an-autoimmune-disease
#18
REVIEW
Adam Aj Al-Diwani, Thomas A Pollak, Sarosh R Irani, Belinda R Lennox
Psychotic disorders are common and disabling. Overlaps in clinical course in addition to epidemiological and genetic associations raise the possibility that autoimmune mechanisms may underlie some psychoses, potentially offering novel therapeutic approaches. Several immune loci including the major histocompatibility complex and B-cell markers CD19 and CD20 achieve genome-wide significance in schizophrenia. Emerging evidence suggests a potential role via neurodevelopment in addition to classical immune pathways...
July 13, 2017: Immunology
https://www.readbyqxmd.com/read/28698711/anti-n-methyl-d-aspartate-receptor-encephalitis-a-severe-potentially-reversible-autoimmune-encephalitis
#19
REVIEW
Cai-Yun Liu, Jie Zhu, Xiang-Yu Zheng, Chi Ma, Xu Wang
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is potentially lethal, but it is also a treatable autoimmune disorder characterized by prominent psychiatric and neurologic symptoms. It is often accompanied with teratoma or other neoplasm, especially in female patients. Anti-NMDAR antibodies in cerebrospinal fluid (CSF) and serum are characteristic features of the disease, thereby suggesting a pathogenic role in the disease. Here, we summarize recent studies that have clearly documented that both clinical manifestations and the antibodies may contribute to early diagnosis and multidisciplinary care...
2017: Mediators of Inflammation
https://www.readbyqxmd.com/read/28684941/-autoimmune-epilepsy-encephalitis-with-autoantibodies-for-epileptologists
#20
Christian G Bien, Martin Holtkamp
Autoimmune encephalitides may account for epilepsies of so far unknown cause. These "autoimmune epilepsies" may respond well to immunotherapy. More than a dozen autoantibodies have been found with this constellation; therefore, broad autoantibody testing of serum-CSF pairs offers the best diagnostic yield. Several particular features raise the suspicion of an autoimmune cause in otherwise unexplained seizure disorders.
May 2017: Epilepsy Currents
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