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https://www.readbyqxmd.com/read/29353399/mri-findings-in-glutamic-acid-decarboxylase-associated-autoimmune-epilepsy
#1
Jason R Fredriksen, Carrie M Carr, Kelly K Koeller, Jared T Verdoorn, Avi Gadoth, Sean J Pittock, Amy L Kotsenas
PURPOSE: Glutamic acid decarboxylase (GAD65) has been implicated in a number of autoimmune-associated neurologic syndromes, including autoimmune epilepsy. This study categorizes the spectrum of MRI findings in patients with a clinical diagnosis of autoimmune epilepsy and elevated serum GAD65 autoantibodies. METHODS: An institutional database search identified patients with elevated serum GAD65 antibodies and a clinical diagnosis of autoimmune epilepsy who had undergone brain MRI...
January 20, 2018: Neuroradiology
https://www.readbyqxmd.com/read/29338905/posterior-reversible-encephalopathy-syndrome-a-rare-childhood-case-with-unconsciousness
#2
Akira Komori, Daisuke Mizu, Koichi Ariyoshi
BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is a condition characterized by seizures, altered consciousness, visual disturbances, and headache. Characteristic findings on neuroimaging include cerebral edema, typically involving the parieto-occipital white matter. PRES has been associated with hypertension, autoimmune disease, and Henoch-Schölein purpura (HSP), but few cases have been reported, and fewer cases of PRES have been reported in children. CASE REPORT: We report the case of a 4-year-old girl who presented with blindness and semi-consciousness...
January 12, 2018: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29336839/effect-of-thymectomy-on-refractory-autoimmune-status-epilepticus
#3
Alberto Vogrig, Giada Pauletto, Enrico Belgrado, Enrico Pegolo, Carla Di Loreto, Veronique Rogemond, Jerome Honnorat, Roberto Eleopra
Refractory status epilepticus (RSE) is an increasingly recognized manifestation of autoimmune encephalitis, which can occur either as a paraneoplastic or non-paraneoplastic disorder. The effect of tumor removal in paraneoplastic status epilepticus has never been explored systematically, although early tumor treatment is usually recommended. In this study, we report clinical, pathological and EEG findings of a patient who developed RSE as one of multiple paraneoplastic manifestations of thymoma and the effect of thymectomy on seizure outcome...
January 9, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29325172/pres-review-of-histological-features
#4
Nicholas Willard, Justin M Honce, B K Kleinschmidt-DeMasters
Posterior reversible encephalopathy syndrome was described in 1996 as a clinical-neuroimaging entity characterized by parieto-occipital watershed-region edema without overt infarction. It has been linked to hypertension, eclampsia, immunosuppressive therapies, infections, and autoimmune disorders. The condition usually has an acute onset, presents with seizures, and ameliorates within days. There have been few neuropathological studies, but in some cases, tissue damage may be more permanent.
February 1, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29317588/-autoimmune-factors-and-epilepsy
#5
Yating Wu, Aihua Liu
Epilepsy is a common chronic brain disorder with multiple causes, and now autoimmune factors are believed to be an independent cause for epilepsy. Accumulating data support an autoimmune basis in patients with antiepileptic drug-resistant seizures. Moreover, systemic autoimmune diseases and epilepsy co-occur frequently. Neural specific autoantibodies can also mediate the process of seizures through different pathways. The diagnosis of autoimmune epilepsy is based on frequency of antiepileptic drug-resistant seizures, the presence of neural specific autoantibodies, inflammatory changes in serum or spinal fluid or on magneticresonanceimaging, the change in electroencephalogram, a personal or family history of autoimmunity...
December 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/29310866/initial-clinical-presentation-of-young-children-with-n-methyl-d-aspartate-receptor-encephalitis
#6
Marion Favier, Bastien Joubert, Géraldine Picard, Véronique Rogemond, Laure Thomas, Sylvain Rheims, Marion Bailhache, Frédéric Villega, Jean-Michel Pédespan, Giulia Berzero, Dimitri Psimaras, Jean-Christophe Antoine, Virginie Desestret, Jérôme Honnorat
Autoimmune encephalitis with anti-N-methyl-d-aspartate receptor autoantibodies (NMDA-R-Abs) is a recently described disease affecting adult and pediatric patients. Symptoms of the disease are now perfectly described in the adult population but the clinical presentation is less known in young children. The aim of the present study was to describe the clinical presentation and the specificities of symptoms presented by young children with NMDA-R-Abs encephalitis to improve diagnosis of this disease, and to compare these to a series of previously published female adult patients...
December 28, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29303953/voltage-gated-potassium-channel-antibody-autoimmune-encephalopathy-presenting-with-isolated-psychosis-in-an-adolescent
#7
David A Kahn
Antibody-mediated encephalopathies associated with serum or cerebrospinal fluid antibodies directed against neuronal structures may present with a multitude of neuropsychiatric syndromes. Although some of the antibody-driven conditions are now well recognized in adults (eg, N-methyl-D-aspartate receptor antibody encephalitis), the spectrum of neuropsychiatric manifestations in the pediatric population is less clear. Psychosis, confusion, catatonia, and additional behavioral changes, along with seizures, encephalopathy, and movement disorders, may be initial manifestations or concurrent features in all age groups...
November 2017: Journal of Psychiatric Practice
https://www.readbyqxmd.com/read/29303952/voltage-gated-potassium-channel-antibody-autoimmune-encephalopathy-presenting-with-isolated-psychosis-in-an-adolescent
#8
Natalie C Pon, Kimberly M Houck, Eyal Muscal, Sindhu A Idicula
Antibody-mediated encephalopathies associated with serum or cerebrospinal fluid antibodies directed against neuronal structures may present with a multitude of neuropsychiatric syndromes. Although some of the antibody-driven conditions are now well recognized in adults (eg, N-methyl-D-aspartate receptor antibody encephalitis), the spectrum of neuropsychiatric manifestations in the pediatric population is less clear. Psychosis, confusion, catatonia, and additional behavioral changes, along with seizures, encephalopathy, and movement disorders, may be initial manifestations or concurrent features in all age groups...
November 2017: Journal of Psychiatric Practice
https://www.readbyqxmd.com/read/29287596/gamma-aminobutyric-acid-b-limbic-encephalitis-and-asystolic-cardiac-arrest-a-case-report
#9
Christopher A Ovens, Angelo Jayamanne, Andrew Duggins
BACKGROUND: Gamma-aminobutyric acid-B receptor autoantibodies are becoming an increasingly recognized contributor to the spectrum of autoimmune limbic encephalitis. They are classically associated with seizures and behavioral disturbance, and may coexist with other autoantibodies. Many are paraneoplastic, most commonly associated with small cell lung cancer. Until now there have been no reports of cardiac dysrhythmias in these patients. CASE PRESENTATION: A 65-year-old Caucasian man presented with multiple seizures, dysarthria and behavioral disturbance of unclear etiology, with associated asystolic cardiac arrest...
December 29, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29282342/-motor-symptoms-of-autoimmune-encephalopathies
#10
Yuichi Tashiro, Hiroshi Takashima
Autoimmune encephalopathies are clinically and immunologically heterogeneous disorders. At least 20 types of autoimmune encephalopathies have been discovered, with the most common type being Hashimoto encephalopathy. In clinical situations, we often observe that patients with autoimmune encephalopathy are misdiagnosed because they exhibit signs similar to those observed in functional psychogenic movement, conversion, or somatoform disorders. We clinically examined over 100 patients with autoimmune encephalopathy...
December 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29260024/unihemispheric-central-nervous-system-vasculitis
#11
Sikawat Thanaviratananich, Bashar Katirji
Patients with primary central nervous system vasculitis (PCNSV) usually manifest with multiple enhancing bilateral hemispheric lesions. We presented an extremely rare clinical course and follow-up of a patient with PCNSV affecting only a single (right) hemisphere. A 33-year-old previously healthy man presented with a left hand clonic seizure followed by a secondary generalized tonic-clonic seizure and dysarthria. MRI brain revealed multiple hyperintense lesions confined to only the right hemisphere with contrast enhancement, involving both white and grey matters...
June 2017: ENeurologicalSci
https://www.readbyqxmd.com/read/29258969/a-case-of-anti-nmda-receptor-encephalitis-revealed-by-insular-epilepsy
#12
Charlotte De Maeseneire, Riem El Tahry, Susana Ferrao Santos
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder of the central nervous system that typically manifests predominantly as a psychiatric disorder. However, other manifestations such as epileptic seizures, abnormal movements, and memory or language complications are not unusual. Here, we report the case of a young man who presented with a new-onset epilepsy, with ictal semiology suggestive of insular involvement; this hypothesis was supported by a PET-CT study. Anti-NMDAR antibodies were found in the CSF, confirming the diagnosis of anti-NMDAR encephalitis...
December 20, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29258968/refractory-chronic-epilepsy-associated-with-neuronal-auto-antibodies-could-perisylvian-semiology-be-a-clue
#13
Lisa Gillinder, Linda Tjoa, Basil Mantzioris, Stefan Blum, Sasha Dionisio
We report a case series of 10 patients with chronic medically refractory antibody-positive autoimmune epilepsy and assess their common clinical features. Immune-mediated seizures are most commonly reported in the context of encephalitis or encephalopathy, with few reports focusing on lone, chronic epilepsy in the outpatient setting. Our aim was to define the potential diagnostic clues that might be present in these cases, leading to consideration of an autoimmune cause of the epilepsy. We performed a retrospective review of all patients presenting to the outpatient department of our unit who underwent autoimmune screening...
December 20, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29237452/molecular-cloning-and-characterization-of-the-family-of-feline-leucine-rich-glioma-inactivated-lgi-genes-and-mutational-analysis-in-familial-spontaneous-epileptic-cats
#14
Yoshihiko Yu, Daisuke Hasegawa, Aki Fujiwara-Igarashi, Yuji Hamamoto, Shunta Mizoguchi, Takayuki Kuwabara, Michio Fujita
BACKGROUND: Leucine-rich glioma-inactivated (LGI) proteins play a critical role in synaptic transmission. Dysfunction of these genes and encoded proteins is associated with neurological disorders such as genetic epilepsy or autoimmune limbic encephalitis in animals and human. Familial spontaneous epileptic cats (FSECs) are the only feline strain and animal model of familial temporal lobe epilepsy. The seizure semiology of FSECs comprises recurrent limbic seizures with or without evolution into generalized epileptic seizures, while cats with antibodies against voltage-gated potassium channel complexed/LGI1 show limbic encephalitis and recurrent limbic seizures...
December 13, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/29208845/epilepsy-among-systemic-lupus-erythematosus-patients-insights-from-a-large-database-analysis
#15
Abdulla Watad, Shmuel Tiosano, Nicola Luigi Bragazzi, Francesco Brigo, Doron Comaneshter, Arnon D Cohen, Howard Amital
OBJECTIVE: Epilepsy is characterized by a relevant epidemiological and clinical burden. In the extant literature, an increased risk of seizures has been described in several inflammatory/autoimmune disorders, including systemic lupus erythematosus (SLE). However, so far, relatively few and small size-based studies have been conducted. We aimed to investigate the link between seizure and SLE utilizing a large sample of subjects and extensive data analysis. METHODS: Patients with SLE were compared with age- and sex-matched controls regarding the prevalence of epilepsy in a cross-sectional study...
December 1, 2017: Neuroepidemiology
https://www.readbyqxmd.com/read/29203057/febrile-infection-related-epilepsy-syndrome-fires-with-super-refractory-status-epilepticus-revealing-autoimmune-encephalitis-due-to-gabaar-antibodies
#16
D Caputo, R Iorio, F Vigevano, L Fusco
BACKGROUND: Febrile infection-related epilepsy syndrome (FIRES) has been described as an epileptic encephalopathy of unknown etiology affecting previously healthy children following febrile illness. Despite large investigations on autoimmune pathogenesis no membrane antibodies has been associated since now. CASE STUDY: We report a 13 years-old girl with negative history for neurological or autoimmune disease that developed at the sixth day of high fever a super-refractory status epilepticus...
November 24, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29195105/lgi1-antibody-encephalitis-detailed-clinical-laboratory-and-radiological-description-of-13-cases-in-china
#17
Dahai Wang, Qinjian Hao, Lingshuang He, Lan He, Qiang Wang
BACKGROUND AND PURPOSE: LGI1 antibody encephalitis is a synaptic autoimmune disorder that was first reported in 2010. To date, LGI1 antibody encephalitis is a widely-recognized disease in neurology and psychiatry. In order to aid clinical recognition of the condition, we analyze the clinical characteristics of 13 Chinese LGI1 antibody encephalitis patients. METHODS: We analyzed clinical features of patients admitted to the West China Hospital who had been diagnosed with LGI1 antibody encephalitis from 2015 to 2017...
November 14, 2017: Comprehensive Psychiatry
https://www.readbyqxmd.com/read/29193473/autoimmune-glial-fibrillary-acidic-protein-astrocytopathy-in-chinese-patients-a-retrospective-study
#18
Youming Long, Junyan Liang, Huiming Xu, Qingmei Huang, Jie Yang, Cong Gao, Wei Qiu, Shaopeng Lin, Xiaohui Chen
OBJECTIVE: We aimed to describe the clinical, radiological, and pathological features of an autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. METHODS: Data from 19 patients with positive GFAP-IgG in cerebrospinal fluid (CSF) were retrospectively analyzed. RESULTS: The main disease manifestations included myelitis (68.4%), headache (63.2%), abnormal vision (63.2%), fever (52.6%), ataxia (36.8%), psychosis (31.6%), dyskinesia (15...
November 29, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29185868/three-case-studies-of-nonparaneoplastic-limbic-encephalitis-nple-in-young-adult-males-onset-course-and-recovery-following-rehabilitation-services
#19
Robert Perna, Lindsey Harik, Ana Arenivas
Limbic encephalitis (LE) is a rare neurological disorder characterized by inflammation of the brain caused by autoimmunity or infection. LE has been a difficult to define and diagnose disorder due to the insidious and nonspecific (e.g., irritability, low mood, short-term memory complaints) presentation of early symptoms, as well as inconsistent findings on neuroimaging, lumbar puncture serum analysis, and electroencephalogram. Seizures, memory problems, and psychiatric disturbance are among the earliest and most prominent clinical features...
November 29, 2017: Applied Neuropsychology. Child
https://www.readbyqxmd.com/read/29160208/a-case-of-post-leptospirosis-autoimmune-epilepsy-presenting-with-sleep-related-hypermotor-seizures
#20
Prashant Makhija, Siby Gopinath, Sudheeran Kannoth, Kurupath Radhakrishnan
This video-illustrated case report concerns a 49-year-old woman who presented with sleep-related hypermotor seizures. The antecedent history of leptospirosis, high frequency of new-onset seizures, presence of an unclassified anti-neuronal antibody, and dramatic response to steroids strongly supported post-infectious immune-mediated pathogenesis in our patient. To the best of our knowledge, post-leptospirosis autoimmune epilepsy presenting as sleep-related hypermotor seizures has not hitherto been reported. [Published with video sequences on www...
November 21, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
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