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https://www.readbyqxmd.com/read/29774053/postherpes-simplex-encephalitis-a-case-series-of-viral-triggered-autoimmunity-synaptic-autoantibodies-and-response-to-therapy
#1
Harry Alexopoulos, Sofia Akrivou, Sotiria Mastroyanni, Maria Antonopoulou, Argirios Dinopoulos, Melpo Giorgi, Kostas Konstantinou, Evangelos Kouremenos, Maria Lariou, Dimitrios Naoumis, Efterpi Pavlidou, Evaggelos Pavlou, Konstantinos Voudris, Panayotis Vlachoyiannopoulos, Marinos C Dalakas
Background: Recent evidence suggests that patients with herpes simplex virus (HSV) encephalitis may relapse because of autoimmunity against the N-methyl-D-aspartate receptor (NMDAR). We present a case series of post-HSV relapsing encephalopathy associated with antibodies to central nervous system (CNS) synaptic antigens. Patient/Methods: Sera and cerebrospinal fluid (CSF) from five patients with HSV encephalitis who relapsed after antiviral therapy were tested for anti-NMDAR, gamma-aminobutyric acid b receptor (GABAbR), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), Leucine-rich, glioma inactivated 1 (LGI1), anti -contactin-associated protein-like 2 (CASPR2) and dipeptidyl-peptidase-like protein-6 (DDPX) antibodies using cell-based assays...
2018: Therapeutic Advances in Neurological Disorders
https://www.readbyqxmd.com/read/29755396/overlapping-autoimmune-syndromes-in-patients-with-glial-fibrillary-acidic-protein-antibodies
#2
Xinguang Yang, Huiming Xu, Meilin Ding, Qingmei Huang, Baikeng Chen, Huacai Yang, Tianni Liu, Youming Long, Cong Gao
Background: Glial fibrillary acidic protein (GFAP) astrocytopathy, an autoimmune central nervous system disorder with a specific GFAP-IgG, often coexists with other antibodies. Objective: The aim of this article was to study overlapping syndromes in autoimmune GFAP astrocytopathy. Methods: Antibody was detected by indirect immunofluorescence assay. Patient data were analyzed retrospectively. Results: Thirty patients with positive GFAP-IgG were included, of whom 10 were defined as overlapping syndrome...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29754014/efficacy-of-antiepileptic-drugs-in-autoimmune-epilepsy-a-systematic-review
#3
REVIEW
Pablo Cabezudo-García, Natalia Mena-Vázquez, Macarena Villagrán-García, Pedro J Serrano-Castro
OBJECTIVE: Review the evidence of the efficacy of AEDs (antiepileptic drugs) in autoimmune epilepsy. MATERIAL AND METHODS: Literature research on Medline and Embase was carried out through January 2018. We included MeSH terms, free text and terms related to "autoimmune epilepsy", "autoimmune encephalitis", "limbic encephalitis", "autoimmune seizures", "antiepileptic drug", "seizure treatment", and "epilepsy treatment"...
May 7, 2018: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/29745347/febrile-infection-related-epilepsy-syndrome-fires-an-overview-of-treatment-and-recent-patents
#4
Kam Lun E Lun Hon, Alexander K C Leung, Alcy R Torres
BACKGROUND: New-onset refractory status epilepticus (NORSE) refers to a clinical presentation in a patient without active epilepsy or other existing relevant neurological disorder, with new onset of refractory status epilepticus in the absence of a clear acute or active structural, metabolic, or toxic cause. Febrile infection-related epilepsy syndrome (FIRES) is a subset of NORSE that requires a febrile infection between 24 hours and 2 weeks prior to the onset of refractory status epilepticus, with or without fever at the onset of status epilepticus, and with no restriction to the age of the patient...
May 8, 2018: Recent Patents on Inflammation & Allergy Drug Discovery
https://www.readbyqxmd.com/read/29742721/coexistence-of-lambert-eaton-myasthenic-syndrome-and-autoimmune-encephalitis-with-anti-crmp5-cv2-and-anti-gabab-receptor-antibodies-in-small-cell-lung-cancer-a-case-report
#5
Hongfang Li, Aimei Zhang, Yanlei Hao, Hongzhi Guan, Zhanyun Lv
RATIONALE: Autoimmune encephalitis and Lambert-Eaton myasthenic syndrome are classic paraneoplastic neurological conditions common in patients with small cell lung cancer. PATIENT CONCERNS: The patient complained of tiredness, fluctuating recent memory loss, and inability to find his home. His family members reported a change in character, irritability, and paranoia. One month later, the patient had 1 grand mal seizure lasting 5 minutes. DIAGNOSIS: The patient was diagnosed with limbic encephalitis combined with Lambert-Eaton myasthenic syndrome...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29733071/symmetrical-and-bilateral-basal-ganglia-calcification-case-series-and-literature-review
#6
Amado Jiménez-Ruiz, Omar Cárdenas-Sáenz, José Luis Ruiz-Sandoval
Introduction: Symmetric, bilateral basal ganglia calcification is rare finding that sometimes occurs asymptomatically. Its prevalence increases with age, and the most affected site is the globus pallidus. Report of cases: A series of seven cases with clinical and imaging diagnosis of basal ganglia calcification, recorded during the 2012 to 2016 period at the Department of Internal Medicine of the Hospital Civil de Guadalajara "Fray Antonio Alcalde, is presented...
2018: Gaceta Médica de México
https://www.readbyqxmd.com/read/29717222/paraneoplastic-limbic-encephalitis-associated-with-lung-cancer
#7
Kaini Shen, Yan Xu, Hongzhi Guan, Wei Zhong, Minjiang Chen, Jing Zhao, Longyun Li, Mengzhao Wang
Paraneoplastic limbic encephalitis (PLE) is a rare autoimmune neurological syndrome observed in lung cancer patients. We retrospectively investigated the clinical characteristics, treatment responses, and prognoses in 16 PLE patients who were subsequently diagnosed with lung cancer. Fifteen patients initially presented with disturbance of consciousness, 13 with disorientation, and 12 with seizures. Thirteen patients had autoantibodies, including eight with gamma aminobutyric acid B receptor (GABAB R) antibodies and eight with Hu antibodies...
May 1, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29683874/time-dependent-decline-in-serum-phenytoin-concentration-with-heightened-convulsive-seizure-risk-by-prolonged-administration-of-fosphenytoin-in-japanese-a-retrospective-study
#8
Yuta Ohno, Takashi Niwa, Keita Hirai, Keiko Suzuki, Yuto Yamada, Yuichi Hayashi, Hideki Hayashi, Akio Suzuki, Yoshinori Itoh
BACKGROUND: Because clinical data to confirm the safety and effectiveness of fosphenytoin, a prodrug of phenytoin, are insufficient, the length of administration of fosphenytoin is restricted. Nevertheless, some cases require fosphenytoin administration for more than a few days. The aim of this study was to retrospectively investigate the serum concentration of phenytoin in adult Japanese patients who received intravenous fosphenytoin therapy for more than 3 days. METHODS: Patients injected with intravenous fosphenytoin for more than 3 days at Gifu University Hospital between January 2012 and September 2014 were enrolled...
April 20, 2018: Therapeutic Drug Monitoring
https://www.readbyqxmd.com/read/29626021/acute-encephalitis-diagnosis-and-management
#9
Mark Ellul, Tom Solomon
Encephalitis, inflammation of the brain, is most commonly caused by a viral infection (especially herpes simplex virus [HSV] type 1 in the UK) although autoimmune causes, such as N-methyl D-aspartate receptor (NMDAR) antibody encephalitis, are increasingly recognised. Most patients present with a change in consciousness level and may have fever, seizures, movement disorder or focal neurological deficits. Diagnosis hinges crucially on lumbar puncture and cerebrospinal fluid (CSF) examination, but imaging and electroencephalography (EEG) may also be helpful...
March 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/29588979/claustrum-hyperintensities-a-potential-clue-to-autoimmune-epilepsy
#10
Claude Steriade, David F Tang-Wai, Timo Krings, Richard Wennberg
In a cohort of 34 patients with autoimmune limbic encephalitis and/or epilepsy, we identified 4 patients exhibiting claustrum fluid-attenuated inversion recovery (FLAIR) hyperintensities. All 4 patients presented with explosive onset of seizures and developed medically intractable epilepsy, and 2 exhibited a marked response to immunotherapy. Associated features included cognitive and behavioral disturbances (4/4), cerebrospinal fluid (CSF) lymphocytic pleocytosis (3/4), and a neural autoantibody (2/4). Electroencephalogram (EEG) features consisted of slow wave activity and epileptiform discharges in frontal and parasagittal regions, where ictal patterns were captured in 1 patient...
December 2017: Epilepsia Open
https://www.readbyqxmd.com/read/29574241/acute-disseminated-encephalomyelitis-a-rare-post-malaria-neurological-complication-case-report-and-review-of-the-literature
#11
REVIEW
Joana Carreira, Maria Isabel Casella, Bianca Branco Ascenção, Nuno Pinto Luis, Ana Catarina Gonçalves, Ana Paula Brito, Joana Estalagem Sá, Mário Parreira, Delfim Lopes, José Poças
There are four neurological complications that can occur after malaria treatment at a time when the patient is aparasitaemic: delayed cerebellar ataxia, acute inflammatory demyelinating polyneuropathy, post-malaria neurological syndrome and acute disseminated encephalomyelitis (ADEM). The authors describe a case of a 54-year-old male who presented with encephalopathy and generalized seizures forty-three days after complete recovery from acute malaria by Plasmodium falciparum. Diagnosis of post-malaria ADEM was made based on the acute onset of the neurological symptoms, characteristic findings in magnetic resonance imaging of the brain and prompt response to steroid therapy...
March 21, 2018: Travel Medicine and Infectious Disease
https://www.readbyqxmd.com/read/29562607/a-cohort-study-comparing-women-with-autism-spectrum-disorder-with-and-without-generalized-joint-hypermobility
#12
Emily L Casanova, Julia L Sharp, Stephen M Edelson, Desmond P Kelly, Manuel F Casanova
Reports suggest comorbidity between autism spectrum disorder (ASD) and the connective tissue disorder, Ehlers-Danlos syndrome (EDS). People with EDS and the broader spectrum of Generalized Joint Hypermobility (GJH) often present with immune- and endocrine-mediated conditions. Meanwhile, immune/endocrine dysregulation is a popular theme in autism research. We surveyed a group of ASD women with/without GJH to determine differences in immune/endocrine exophenotypes. ASD women 25 years or older were invited to participate in an online survey...
March 17, 2018: Behavioral Sciences
https://www.readbyqxmd.com/read/29524102/neurological-autoantibodies-in-drug-resistant-epilepsy-of-unknown-cause
#13
Mehmet Tecellioglu, Ozden Kamisli, Suat Kamisli, Fatma Ebru Yucel, Cemal Ozcan
BACKGROUND: Autoimmune epilepsy is a rarely diagnosed condition. Recognition of the underlying autoimmune condition is important, as these patients can be resistant to antiepileptic drugs. AIMS: To determine the autoimmune and oncological antibodies in adult drug-resistant epilepsy of unknown cause and identify the clinical, radiological, and EEG findings associated with these antibodies according to data in the literature. METHODS: Eighty-two patients with drug-resistant epilepsy of unknown cause were prospectively identified...
March 9, 2018: Irish Journal of Medical Science
https://www.readbyqxmd.com/read/29523913/-autoimmune-encephalitis-with-psychotic-symptoms-diagnostics-warning-signs-and-practical-approach
#14
REVIEW
J Steiner, H Prüß, S Köhler, A Hasan, P Falkai
Despite intensive research, a precise cause of schizophrenic and schizoaffective disorders has not yet been identified. Therefore, psychiatric diagnoses are still made based on clinical ICD-10/DSM‑5 criteria and not on any objective markers; however, various causes or pathophysiological processes may ultimately lead to similar symptoms. An important task for the future of psychiatry is to identify disease subtypes with a distinct pathophysiology to develop more specific and causally acting therapies. A new diagnostic entity has become established in clinical neurology and psychiatry in recent years: autoimmune encephalitis with psychotic symptoms caused by specific antineuronal antibodies has been identified as a rare but potentially treatable cause of psychotic disorders; however, these inflammatory brain diseases are not reliably detected by routine psychiatric diagnostics...
May 2018: Der Nervenarzt
https://www.readbyqxmd.com/read/29513076/occult-teratoma-in-a-case-of-n-methyl-d-aspartate-receptor-encephalitis
#15
Anita Lwanga, David O Kamson, Tiffany E Wilkins, Vinny Sharma, Jefree J Schulte, James Miller, Ikram Hassan, Ricardo R Lastra
N-methyl-D-aspartate receptor encephalitis (NMDARe) is one of 13 autoimmune-mediated encephalitides that have been discovered over the last decade. This case report describes the course of a 26-year-old female who presented with new-onset seizures and insomnia, complicated by encephalitis. The initial workup ruled out common causes of encephalitis, while a transvaginal ultrasound (TVUS), and computed tomography (CT) scans of the chest, abdomen, and pelvis did not identify a mass. Based on the suspicion that she may have autoimmune encephalitis, the patient was treated with intravenous immunoglobulins and plasma exchange, but continued to deteriorate...
January 1, 2018: Neuroradiology Journal
https://www.readbyqxmd.com/read/29504979/autoimmune-encephalitis-positive-for-both-anti-%C3%AE-aminobutyric-acid-b-receptor-and-anticollapsin-response-mediator-protein-5-antibodies-a-case-report
#16
Junyang Xia, Xiaoming Yin, Mingqin Zhu, Jie Cao, Xiaonan Song
RATIONALE: Autoimmune encephalitis (AE) is a heterogeneous group of recently identified disorders. Despite severe and even prolonged neurologic deficits, dramatic improvements may occur with proper immunotherapy in some patients with AE. Antineuronal antibodies have been discovered in patients' serum and cerebrospinal fluid (CSF). However, AE with multiple antineuronal antibodies is rare. To date, there are no published reports of AE with both anti-γ-aminobutyric acid B receptor (GABABR) and anticollapsin response-mediator protein 5 (CV2) antibodies...
January 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29499442/autoimmune-gfap-astrocytopathy-after-viral-encephalitis-a-case-report
#17
Jie Li, Yan Xu, Haitao Ren, Yicheng Zhu, Bin Peng, Liying Cui
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a novel autoimmune disease. This is the first report of a case of autoimmune GFAP astrocytopathy after herpes simplex viral encephalitis (HSVE). A 35-year-old female patient presented with a combination of headache, fever, seizure and psychiatric/behavioral abnormalities. She had GFAP-IgG in both serum and cerebrospinal fluid (CSF). Magnetic resonance imaging (MRI) with gadolinium-enhancement revealed linear enhancement oriented radially to the ventricles...
April 2018: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29499358/the-pro-apoptotic-protein-bmf-co-operates-with-bim-and-puma-in-neuron-death-induced-by-%C3%AE-amyloid-or-ngf-deprivation
#18
Rumana Akhter, Suraiya Saleem, Akash Saha, Subhas Chandra Biswas
The pro-apoptotic Bcl-2 homology 3 domain only (BH3-only) proteins are central regulators of cell death in various physiological and pathological conditions, including Alzheimer's disease (AD). Bcl-2 modifying factor (Bmf) is one such BH3-only protein that is implicated in various death paradigms such as anoikis, seizures, cancer and autoimmunity. It also co-operates with other BH3-only proteins such as Bim in various death paradigms. However, its role in neurodegeneration is under-investigated. Here, we report for the first time the essential role of Bmf and its co-operativity with direct activator BH3-only proteins Bim and Puma in neuron death induced by beta-amyloid (Aβ) toxicity or NGF deprivation...
April 2018: Molecular and Cellular Neurosciences
https://www.readbyqxmd.com/read/29468181/seizure-semiology-an-important-clinical-clue-to-the-diagnosis-of-autoimmune-epilepsy
#19
Rui-Juan Lv, Hai-Tao Ren, Hong-Zhi Guan, Tao Cui, Xiao-Qiu Shao
Objective: The purpose of this study is to analyze the seizure semiologic characteristics of patients with autoimmune epilepsy (AE) and describe the investigation characteristics of AE using a larger sample size. Methods: This observational retrospective case series study was conducted from a tertiary epilepsy center between May 2014 and March 2017. Cases of new-onset seizures were selected based on laboratory evidence of autoimmunity. At the same time, typical mesial temporal lobe epilepsy (MTLE) patients with hippocampal sclerosis (HS) were recruited as the control group from the subjects who underwent presurgical evaluation during the same period...
February 2018: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29457123/general-anesthesia-with-propofol-for-ovarian-teratoma-excision-associated-with-anti-n-methyl-d-aspartate-receptor-encephalitis
#20
Masami Sato, Hiroaki Yasumoto, Toshiyuki Arai
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder caused by production of anti-NMDAR antibodies that is often associated with ovarian teratoma and exhibits various manifestations including psychiatric symptoms, seizures, hypoventilation, and autonomic nerve instability. Patients with this disorder who receive early surgical tumor resection along with immunotherapy have better outcome than the rest of the patients. To establish an anesthetic plan, it is important to understand the pharmacological interaction between the anesthetic agents and the disabled NMDAR, because NMDAR is one of the major sites of action for commonly-used anesthetic agents...
2018: JA Clinical Reports
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