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Autoimmune encephalitis

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https://www.readbyqxmd.com/read/28345736/-kleine-levin-syndrome-differential-diagnosis-in-recurrent-encephalitic-syndromes-in-adolescence
#1
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
#2
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 14, 2017: Médecine et Maladies Infectieuses
https://www.readbyqxmd.com/read/28306571/cns-syndromes-associated-with-antibodies-against-metabotropic-receptors
#3
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
#4
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
#5
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
#6
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
https://www.readbyqxmd.com/read/28283100/antecedent-anti-nmda-receptor-encephalitis-in-two-patients-with-multiple-sclerosis
#7
A Baheerathan, W J Brownlee, D T Chard, K Shields, R Gregory, S A Trip
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder characterised by psychiatric symptoms, movement disorder and seizures often evolving into a severe encephalopathy. An overlap has recently been recognised between anti-NMDAR encephalitis and inflammatory demyelinating disorders, particularly neuromyelitis optical spectrum disorder (NMOSD). In this case report, we describe two patients with an initial presentation consistent with anti-NMDAR encephalitis who have subsequently developed relapsing-remitting multiple sclerosis (MS) and discuss the literature pertaining to potential overlap between NMDAR encephalitis and inflammatory demyelinating disorders...
February 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28282627/anti-nmdar-encephalitis-followed-by-seropositive-neuromyelitis-optica-spectrum-disorder-a-case-report-and-literature-review
#8
REVIEW
Ye Ran, Lu Wang, Fangfang Zhang, Ran Ao, Zhao Dong, Shengyuan Yu
Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory central nervous system syndrome, and encephalitis associated with anti-N-methyl-d-aspartate receptor (NMDAR) antibodies is an autoimmune encephalopathy. A patient with both diseases, separately or simultaneously, is rare as a clinical phenomenon, but cannot be ignored. We report the clinical characteristics and imaging features of a special case with anti-NMDAR encephalitis followed by NMOSD. We subsequently reviewed the English language literature about demyelinating disorders with anti-NMDAR encephalitis...
February 27, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28272206/limbic-encephalitis-associated-with-anti-nh2-terminal-of-%C3%AE-enolase-antibodies-a-clinical-subtype-of-hashimoto-encephalopathy
#9
Toru Kishitani, Akiko Matsunaga, Masamichi Ikawa, Kouji Hayashi, Osamu Yamamura, Tadanori Hamano, Osamu Watanabe, Keiko Tanaka, Yasunari Nakamoto, Makoto Yoneda
Several types of autoantibodies have been reported in autoimmune limbic encephalitis (LE), such as antibodies against the voltage-gated potassium channel (VGKC) complex including leucine-rich glioma inactivated 1 (LGI1). We recently reported a patient with autoimmune LE and serum anti-NH2-terminal of α-enolase (NAE) antibodies, a specific diagnostic marker for Hashimoto encephalopathy (HE), who was diagnosed with HE based on the presence of antithyroid antibodies and responsiveness to immunotherapy. This case suggests that LE patients with antibodies to both the thyroid and NAE could be diagnosed with HE and respond to immunotherapy...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28268073/vaccinations-in-early-life-are-not-associated-with-development-of-islet-autoimmunity-in-type-1-diabetes-high-risk-children-results-from-prospective-cohort-data
#10
Andreas Beyerlein, Andreas N Strobl, Christiane Winkler, Michaela Carpus, Annette Knopff, Ewan Donnachie, Donna P Ankerst, Anette-G Ziegler
AIMS/HYPOTHESIS: Vaccinations in early childhood potentially stimulate the immune system and may thus be relevant for the pathogenesis of autoimmune diseases such as type 1 diabetes (T1D). We determined the association of vaccination burden with T1D-associated islet autoimmunity in children with high familial risk followed prospectively from birth. METHODS: A total of 20,570 certified vaccination records from 1918 children were correlated with time to onset of T1D-associated islet autoimmunity using Cox regression, considering multiple time periods up until age two years and vaccination types, and adjusting for HLA genotype, sex, delivery mode, season of birth, preterm delivery and maternal T1D status...
March 3, 2017: Vaccine
https://www.readbyqxmd.com/read/28262035/lesions-of-the-posterior-limb-of-the-internal-capsule-in-neuromyelitis-optica-spectrum-disorder
#11
Youming Long, Linzhan Wu, Rong Zhong, Xiaoming Ouyang, Junyan Liang, Cong Gao, Xiaohui Chen, Wei Qiu, Yanyu Chang, Zhanhang Wang, Jinlong Ye
Posterior limb of the internal capsule lesions (PLICL) are one of the MRI features of neuromyelitis optica spectrum disorder (NMOSD). However, there is no evidence that such lesions are pathogenically related to NMOSD. We retrospectively analyzed features of PLICL in NMOSD, and other central nervous system inflammatory disorders, in 561 patients. We also examined the pathological samples of six patients. Of the 561 patients investigated, PLICL were found in 65 patients (11.6%). Lesions were bilateral in 26 cases (40%) and unilateral in 39 cases (60%)...
March 6, 2017: Neurological Research
https://www.readbyqxmd.com/read/28261116/red-flags-clinical-signs-for-identifying-autoimmune-encephalitis-in-psychiatric-patients
#12
Julia Herken, Harald Prüss
Autoimmune mechanisms causing diverse psychiatric symptoms are increasingly recognized and brought about a paradigm shift in neuropsychiatry. Identification of underlying antibodies against neuronal ion channels or receptors led to the speculation that a number of patients go misdiagnosed with a primary psychiatric disease. However, there is no clear consensus which clinical signs in psychiatric patients should prompt further investigations including measurement of anti-neuronal autoantibodies. We therefore aimed to analyze the presenting symptoms in patients with autoimmune encephalitis and the time between symptom onset and initiation of antibody diagnostics...
2017: Frontiers in Psychiatry
https://www.readbyqxmd.com/read/28259863/-review-of-the-psychiatric-aspects-of-anti-nmda-n-methyl-d-aspartic-acid-receptor-encephalitis-case-report-and-our-plans-for-a-future-study
#13
Levente Herman, Ildiko Reka Zsigmond, Laszlo Peter, Janos M Rethelyi
Anti-NMDAR (N-methyl-D-aspartic acid receptor) encephalitis, first described in 2007, is a rare, autoimmune limbic encephalitis. In half of the cases anti-NMDAR antibodies are paraneoplastic manifestations of an underlying tumor (mostly ovarian teratoma). In the early stage of the disease psychiatric symptoms are prominent, therefore 60-70% of the patients are first treated in a psychiatric department. In most of the cases, typical neurological symptoms appear later. Besides the clinical picture and typical symptoms, verifying presence of IgG antibodies in the serum or CSF is necessary to set up the diagnosis...
December 2016: Neuropsychopharmacologia Hungarica
https://www.readbyqxmd.com/read/28258904/a-simplified-approach-to-encephalitis-and-its-mimics-key-clinical-decision-points-in-the-setting-of-specific-imaging-abnormalities
#14
Colin D McKnight, Aine M Kelly, Myria Petrou, Anna E Nidecker, Matthew T Lorincz, Duaa K Altaee, Stephen S Gebarski, Bradley Foerster
RATIONALE AND OBJECTIVES: Infectious encephalitis is a relatively common cause of morbidity and mortality. Treatment of infectious encephalitis with antiviral medication can be highly effective when administered promptly. Clinical mimics of encephalitis arise from a broad range of pathologic processes, including toxic, metabolic, neoplastic, autoimmune, and cardiovascular etiologies. These mimics need to be rapidly differentiated from infectious encephalitis to appropriately manage the correct etiology; however, the many overlapping signs of these various entities present a challenge to accurate diagnosis...
February 28, 2017: Academic Radiology
https://www.readbyqxmd.com/read/28256379/outcome-of-childhood-onset-epilepsy-from-adolescence-to-adulthood-transition-issues
#15
REVIEW
R Nabbout, D M Andrade, N Bahi-Buisson, H Cross, I Desquerre, O Dulac, T Granata, E Hirsch, V Navarro, L Ouss, P L Pearl, D Schmidt, E Thiele, P R Camfield, C S Camfield
This is the second of three papers that summarize the second symposium on Transition in Epilepsies held in Paris in June 2016. This paper addresses the outcome for some particularly challenging childhood-onset epileptic disorders with the goal of recommending the best approach to transition. We have grouped these disorders in five categories with a few examples for each. The first group includes disorders presenting in childhood that may have late- or adult-onset epilepsy (metabolic and mitochondrial disorders)...
February 27, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28256369/anti-hu-paraneoplastic-brainstem-encephalitis-caused-by-a-pancreatic-neuroendocrine-tumor-presenting-with-central-hypoventilation
#16
Marc Najjar, Andrew Taylor, Surbhi Agrawal, Tito Fojo, Alexander E Merkler, Marc K Rosenblum, Laura Lennihan, Michael D Kluger
Paraneoplastic neurological syndromes are rare autoimmune manifestations of malignancies associated with specific antibodies. Anti-Hu associated brainstem encephalitis, a well-described syndrome, usually presents subacutely with preferential involvement of the medulla. Anti-Hu antibodies target intraneuronal antigens and are therefore highly correlated with neurological syndromes when present concomitantly with a neoplasm. Reported is a case of anti-Hu brainstem encephalitis associated with a pancreatic neuroendocrine tumor (PNET) presenting with central hypoventilation...
February 27, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28251511/herpes-simplex-encephalitis-an-update
#17
REVIEW
John W Gnann, Richard J Whitley
PURPOSE OF REVIEW: The goal of this review is to provide an update on current thinking regarding herpes simplex encephalitis (HSE), emphasizing new information about pathogenesis, diagnosis, and immune responses. Specific questions to be addressed are the following: (1) Is there a genetic predisposition to HSE? (2) What clinical approaches have the greatest impact on improving the long-term outcomes in patients with HSE? And (3) are there immune-mediated mechanisms that may account for relapsing HSE? RECENT FINDINGS: Toll-like receptor 3 (TLR 3) plays an important role in innate immune responses, including generation of interferons...
March 2017: Current Infectious Disease Reports
https://www.readbyqxmd.com/read/28248701/autoimmune-encephalitis-with-anti-leucine-rich-glioma-inactivated-1-or-anti-contactin-associated-protein-like-2-antibodies-formerly-called-voltage-gated-potassium-channel-complex-antibodies
#18
Anna E M Bastiaansen, Agnes van Sonderen, Maarten J Titulaer
PURPOSE OF REVIEW: Twenty years since the discovery of voltage-gated potassium channel (VGKC)-related autoimmunity; it is currently known that the antibodies are not directed at the VGKC itself but to two closely associated proteins, anti-leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (Caspr2). Antibodies to LGI1 and Caspr2 give well-described clinical phenotypes. Anti-LGI1 encephalitis patients mostly have limbic symptoms, and anti-Caspr2 patients have variable syndromes with both central and peripheral symptoms...
February 28, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28245617/the-diverse-roles-of-microglia-in-the-neurodegenerative-aspects-of-central-nervous-system-cns-autoimmunity
#19
REVIEW
Kaitlyn K Thompson, Stella E Tsirka
Autoimmune diseases of the central nervous system (CNS) involve inflammatory components and result in neurodegenerative processes. Microglia, the resident macrophages of the CNS, are the first responders after insults to the CNS and comprise a major link between the inflammation and neurodegeneration. Here, we will focus on the roles of microglia in two autoimmune diseases: the prevalent condition of multiple sclerosis (MS) and the much rarer Rasmussen's encephalitis (RE). Although there is an abundance of evidence that microglia actively contribute to neuronal damage in pathological states such as MS and RE, there is also evidence of important reparative functions...
February 25, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28243168/relapsing-polychondritis-presented-with-encephalitis-followed-by-brain-atrophy
#20
Suk-Won Ahn, Moo-Seok Park, Hae-Bong Jeong, Oh-Sang Kwon, Byung-Nam Yoon, Hee Sung Kim, Sang Tae Choi
Relapsing polychondritis (RP) is a rare autoimmune disease that is characterized by inflammatory reaction of unknown etiology and destruction of cartilaginous structures. Characteristic symptoms of this disease include cartilage inflammation of the ear, nose, larynx, trachea, bronchi, joints, eyes, heart and skin. Concomitance with neurologic symptom is very rare in RP, and the detailed underlying mechanism of neurological involvement associated with RP is not fully understood. We herein described an unusual recurrent case of inflammatory brain lesions associated with RP, with attention to clinical manifestations, autoimmune disease involvement, and therapeutic effects...
February 2017: Experimental Neurobiology
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