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https://www.readbyqxmd.com/read/28926420/seronegative-paraneoplastic-limbic-encephalitis-associated-with-thymoma
#1
Jaime Toro, David Cuellar-Giraldo, Alejandra Duque, Karla Minota, Jorge Patiño, Manuel García
Paraneoplastic limbic encephalitis is an autoimmune syndrome characterized by the acute or subacute onset of encephalopathy, memory loss, confusion, temporal lobe seizures, and behavioral and mood changes. Although most patients with paraneoplastic limbic encephalitis have antineuronal antibodies, advances in the field now permit the diagnosis without autoantibody test results. In this case illustrating the new diagnostic criteria, we report a 70-year-old woman who was brought to the emergency room after the acute onset of cognitive impairment, altered mental status, and choreoathetoid movements...
September 2017: Cognitive and Behavioral Neurology: Official Journal of the Society for Behavioral and Cognitive Neurology
https://www.readbyqxmd.com/read/28923528/arterial-spin-labeling-hyperperfusion-in-rasmussen-s-encephalitis-is-it-due-to-focal-brain-inflammation-or-a-post-ictal-phenomenon
#2
Savith Kumar, Chinmay P Nagesh, Bejoy Thomas, Ashalatha Radhakrishnan, Ramshekhar N Menon, Chandrasekharan Kesavadas
BACKGROUND AND PURPOSE: The study evaluated the utility of Arterial spin labeling (ASL) perfusion imaging in Rasmussen's Encephalitis (RE). MATERIAL AND METHODS: The hospital electronic database was searched using the search words "encephalitis," "autoimmune encephalitis" and "Rasmussen's encephalitis" for the period of 1 Jan 2015 to 31 Jan 2017. Clinically diagnosed cases of RE for which epilepsy protocol magnetic resonance imaging (MRI) with perfusion imaging (ASL) performed on a 3T scanner were retrieved...
September 15, 2017: Journal of Neuroradiology. Journal de Neuroradiologie
https://www.readbyqxmd.com/read/28919330/limbic-encephalitis-with-lgi1-antibodies-in-a-14-year-old-boy
#3
M Schimmel, M C Frühwald, C G Bien
Limbic encephalitis (LE) with antibodies against leucine-rich glioma inactivated protein 1 (LGI1) is an auto-antibody mediated disorder with characteristic symptoms as dysfunction of memory, faciobrachial dystonic seizures and neuropsychiatric symptoms as emotional lability. Limbic encephalitis with LGI1 antibodies has been known so far as a disease of adults. We describe the case of a 14-year-old boy presenting with typical dysfunction of memory and LGI1 antibodies. To the best of our knowledge, this is the youngest patient with LGI1 antibody mediated limbic encephalitis described so far...
September 2, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28914199/clinical-applications-of-intravenous-immunoglobulins-in-child-neurology
#4
Maria Gogou, Efimia Papadopoulou-Alataki, Martha Spilioti, Sofia Alataki, Athanasios Evangeliou
BACKGROUND: While there are guidelines for the use of intravenous immunoglobulins in children with Guillain-Barre syndrome and myasthenia gravis based on high-level evidence studies, data is fewer for the majority of neurologic disorders in this age group. Neuronal antibodies are detected in children with seizures of autoimmune etiology. Intravenous immunoglobulins with their broad immunomodulatory mechanism of action could be ideally effective in different forms of immune-dysregulated intractable epilepsies such as autoimmune epilepsy and autoimmune Rasmussen encephalitis...
September 15, 2017: Current Pharmaceutical Biotechnology
https://www.readbyqxmd.com/read/28910681/the-imaging-spectrum-of-posterior-reversible-encephalopathy-syndrome-a-pictorial-review
#5
REVIEW
Emily Brady, Neal S Parikh, Babak B Navi, Ajay Gupta, Andrew D Schweitzer
Posterior reversible encephalopathy syndrome (PRES) is characterized by the acute onset of neurologic symptoms (headache, altered mental status, visual changes, seizures) with accompanying vasogenic edema on brain imaging. Risk factors for PRES include infection, uremia, malignancy, autoimmune disorders, the peripartum state and hypertension. PRES is classically described as being posterior (i.e. parieto-occipital) but radiologic variants are increasingly recognized. This pictorial review demonstrates the heterogeneity of the different radiologic presentations of PRES in reference to lesion distribution, hemorrhage, diffusion restriction, contrast enhancement, and other associated findings...
August 30, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28904568/pediatric-autoimmune-encephalitis
#6
REVIEW
Massimo Barbagallo, Giovanna Vitaliti, Piero Pavone, Catia Romano, Riccardo Lubrano, Raffaele Falsaperla
Autoimmune (antibody mediated) encephalitis (AE) is emerging as a more common cause of pediatric encephalopathy than previously thought. The autoimmune process may be triggered by an infection, vaccine, or occult neoplasm. In the latter case, onconeural autoantibodies are directed against intracellular neuronal antigens, but a recent heterogeneous group of encephalitic syndromes has been found not to have underlying tumor but is associated with autoantibodies to the neuronal surface or synaptic antigens. Neuropsychiatric symptoms are very common in autoimmune encephalopathy; as a result, affected children may be initially present to psychiatrists...
April 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28900675/bone-disease-in-connective-tissue%C3%A2-disease-systemic-lupus-erythematosus
#7
REVIEW
Irene E M Bultink
This article reviews recent advances in the research of the mechanisms of bone loss, as well as clinical features, economic impact and therapeutic implications of osteoporosis and fractures in patients with systemic lupus erythematosus (SLE) as an illustration of bone disease in a complex systemic autoimmune connective tissue disease. Recent studies demonstrated an increased incidence of osteoporosis and peripheral and vertebral fractures in patients with SLE. The aetiology of bone loss in SLE is multifactorial, including clinical osteoporosis risk factors, systemic inflammation, serological factors, metabolic factors, hormonal factors, possibly genetic factors and medication-induced adverse effects...
September 12, 2017: Calcified Tissue International
https://www.readbyqxmd.com/read/28884051/anti-n-methyl-d-aspartate-receptor-nmdar-encephalitis-in-association-with-ovarian-teratoma
#8
Javaad Ahmad, Muhammad Saad Sohail, Amina Khan, Ahmed H Qavi, Pramod Gaudel, Mehr Zahid, Salman Assad
Anti-N-methyl-D-aspartate-Receptor (NMDAR) encephalitis is an autoimmune disorder with a multifaceted presentation that involves memory deficits, psychiatric symptoms, and autonomic instability. This case report describes the classic presentation of Anti-NMDAR encephalitis and highlights its association with ovarian teratomas. We present a 26 -year-old female who came in with new onset seizures and altered mentation who subsequently developed automatism. Electroencephalograms (EEG) showed left frontal spikes and right temporal delta activity...
July 5, 2017: Curēus
https://www.readbyqxmd.com/read/28878050/antibody-associated-cns-syndromes-without-signs-of-inflammation-in-the-elderly
#9
Domingo Escudero, Mar Guasp, Helena Ariño, Carles Gaig, Eugenia Martínez-Hernández, Josep Dalmau, Francesc Graus
OBJECTIVE: To report the CNS syndromes of patients ≥60 years of age with antibodies against neuronal surface antigens but no evidence of brain MRI and CSF inflammatory changes. METHODS: This was a retrospective clinical analysis of patients with antibodies against neuronal surface antigens who fulfilled 3 criteria: age ≥60 years, no inflammatory abnormalities in brain MRI, and no CSF pleocytosis. Antibodies were determined with reported techniques. RESULTS: Among 155 patients ≥60 years of age with neurologic syndromes related to antibodies against neuronal surface antigens, 35 (22...
September 6, 2017: Neurology
https://www.readbyqxmd.com/read/28857066/hypoparathyroidism
#10
REVIEW
Michael Mannstadt, John P Bilezikian, Rajesh V Thakker, Fadil M Hannan, Bart L Clarke, Lars Reijnmark, Deborah M Mitchell, Tamara J Vokes, Karen K Winer, Dolores M Shoback
Hypoparathyroidism is a disease characterized by inadequately low circulating concentrations of parathyroid hormone (PTH) resulting in low calcium levels and increased phosphate levels in the blood. Symptoms of the disease result from increased neuromuscular irritability caused by hypocalcaemia and include tingling, muscle cramps and seizures. The most common cause of the disease is inadvertent removal of, or injury to, the parathyroid glands during neck surgery, followed by genetic, idiopathic and autoimmune aetiologies...
August 31, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28841275/neuropsychiatric-manifestations-of-pediatric-nmda-receptor-autoimmune-encephalitis-a-case-series-from-a-tertiary-care-center-in-india
#11
Salah Basheer, Madhu Nagappa, Anita Mahadevan, Parayil Sankaran Bindu, Arun B Taly, Satish Chandra Girimaji
Objective: Although psychiatric manifestations are one of the most common presentations of pediatric N-methyl-D-aspartate receptor (NMDAR) encephalitis, there is a lack of studies that characterize psychiatric aspects of this disorder. This study was designed to address this gap. Methods: Initial clinical presentations including psychiatric symptoms, treatment details, and outcome with respect to psychiatric symptoms were collected from medical case records of children aged less than 18 years with seropositive NMDAR encephalitis from a single tertiary care center (May 2010-November 2016)...
August 17, 2017: Primary Care Companion to CNS Disorders
https://www.readbyqxmd.com/read/28829986/neurobiology-of-autoimmune-encephalitis
#12
REVIEW
Masaki Fukata, Norihiko Yokoi, Yuko Fukata
Autoimmune encephalitis presenting with amnesia, seizures, and psychosis is highly topical in basic and clinical neuroscience. Recent studies have identified numerous associated autoantibodies, targeting cell-surface synaptic proteins including neurotransmitter receptors (e.g. NMDA receptors (NMDARs)) and a secreted protein, LGI1. In vitro and in vivo analyses of the influence of the autoantibodies have begun to clarify their causal roles. Of particular interest is the generation of recombinant monoclonal antibodies from patients' B cells with anti-NMDAR encephalitis...
August 19, 2017: Current Opinion in Neurobiology
https://www.readbyqxmd.com/read/28804482/risk-factors-for-intensive-care-unit-admission-in-patients-with-autoimmune-encephalitis
#13
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
#14
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...
September 2017: Pediatrics
https://www.readbyqxmd.com/read/28789769/-autoimmune-encephalitis-presenting-with-drug-resistant-status-epilepticus
#15
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/28770118/new-onset-insomnia-in-a-pediatric-patient-a-case-of-anti-nmda-receptor-encephalitis
#16
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/28766694/musicogenic-reflex-seizures-in-epilepsy-with-glutamic-acid-decarbocylase-antibodies
#17
M Falip, L Rodriguez-Bel, S Castañer, J Miro, S Jaraba, J Mora, J Bas, M Carreño
BACKGROUND: Musicogenic reflex seizures (MRS) are a rare form of seizures described in patients with temporal lobe epilepsy (TLE), mainly of unknown etiology. Epilepsy with antibodies against glutamic acid decarboxylase (GAD-ab) is a form of autoimmune epilepsy for which no specific semiology has been described. AIM OF THE STUDY: To retrospectively review the incidence of MRS in the general epileptic population and in the series of patients with epilepsy and GAD-ab and to describe its clinical and paraclinical characteristics...
August 2, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/28761904/iglon5-antibody-neurological-accompaniments-and-outcomes-in-20-patients
#18
Josephe A Honorat, Lars Komorowski, Keith A Josephs, Kai Fechner, Erik K St Louis, Shannon R Hinson, Sabine Lederer, Neeraj Kumar, Avi Gadoth, Vanda A Lennon, Sean J Pittock, Andrew McKeon
OBJECTIVE: To describe the phenotypes, treatment response, and outcome of IgLON5 autoimmunity. METHODS: Archived serum and CSF specimens from 367 patients known to harbor unclassified antibodies which stained neural synapses diffusely (mimicking amphiphysin-IgG) were reevaluated by indirect immunofluorescence assay (IFA) using a composite of mouse tissues and recombinant IgLON5-transfected cell-based assay (CBA, Euroimmun). RESULTS: Available specimens (serum, 25; CSF, 9) from 26/367 patients (7%) had identical IFA appearance and robust IgLON5 CBA positivity...
September 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28750305/progressive-hippocampal-sclerosis-after-viral-encephalitis-potential-role-of-nmda-receptor-antibodies
#19
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/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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