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https://www.readbyqxmd.com/read/29413523/anti-nmda-receptor-encephalitis-clinical-features-and-basic-mechanisms
#1
David R Lynch, Amy Rattelle, Yi Na Dong, Kylie Roslin, Amy J Gleichman, Jessica A Panzer
In slightly more than 10 years, anti-NMDA receptor (NMDAR) encephalitis has changed from a rare paraneoplastic syndrome to the most common cause of nonviral encephalitis. It presents fulminantly with progressive psychosis, seizures, and autonomic dysfunction, leading to death if untreated. However, rapid recognition and treatment can lead to survival and a return to baseline levels of functioning in many patients. While initially associated with ovarian teratomas, it is now associated with other tumors and can reflect a postviral event...
2018: Advances in Pharmacology
https://www.readbyqxmd.com/read/29408771/headache-in-mitochondrial-disorders
#2
REVIEW
Josef Finsterer, Sinda Zarrouk-Mahjoub
Headache is a prominent feature in mitochondrial disorders (MIDs) but no comprehensive overview is currently available. This review aims at summarising and discussing findings concerning type, frequency, pathogenesis, and treatment of headache in MIDs. The most frequent headache types in MIDs are migraine and migraine-like headache (MLH). MLH is classified as secondary headache. More rarely, tension-type headache, trigemino-autonomic headache, or different secondary headaches can be found. Migraine or MLH may manifest with or without aura...
January 26, 2018: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/29370977/severe-dysautonomia-as-a-main-feature-of-anti-gad-encephalitis-report-of-a-paediatric-case-and-literature-review
#3
Nedia Ben Achour, Thouraya Ben Younes, Ibtihel Rebai, Melika Ben Ahmed, Ichraf Kraoua, Ilhem Ben Youssef-Turki
INTRODUCTION: Anti-glutamic acid decarboxylase (anti-GAD65) antibodies are a rare cause of autoimmune encephalitis. This entity is mainly recognized in adults and very few cases were reported in children. We report on a paediatric case of anti-GAD encephalitis with severe presentation and uncontrollable dysautonomia. CASE STUDY: A 9-year-old girl was referred to our department for refractory seizures and behavioral disturbances. Brain magnetic resonance imaging (MRI) was normal...
January 12, 2018: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29368690/bad-and-katp-channels-regulate-neuron-excitability-and-epileptiform-activity
#4
Juan Ramón Martínez-François, María Carmen Fernández-Agüera, Nidhi Nathwani, Carolina Lahmann, Veronica L Burnham, Nika N Danial, Gary Yellen
Brain metabolism can profoundly influence neuronal excitability. Mice with genetic deletion or alteration of Bad (BCL-2 agonist of cell death) exhibit altered brain-cell fuel metabolism, accompanied by resistance to acutely induced epileptic seizures; this seizure protection is mediated by ATP-sensitive potassium (KATP) channels. Here we investigated the effect of BAD manipulation on KATP channel activity and excitability in acute brain slices. We found that BAD's influence on neuronal KATP channels was cell-autonomous and directly affected dentate granule neuron (DGN) excitability...
January 25, 2018: ELife
https://www.readbyqxmd.com/read/29364393/autoimmune-encephalitis-a-review-of-diagnosis-and-treatment
#5
Lívia Almeida Dutra, Fabiano Abrantes, Fabio Fieni Toso, José Luiz Pedroso, Orlando Graziani Povoas Barsottini, Romana Hoftberger
Autoimmune encephalitis (AIE) is one of the most common causes of noninfectious encephalitis. It can be triggered by tumors, infections, or it may be cryptogenic. The neurological manifestations can be either acute or subacute and usually develop within six weeks. There are a variety of clinical manifestations including behavioral and psychiatric symptoms, autonomic disturbances, movement disorders, and seizures. We reviewed common forms of AIE and discuss their diagnostic approach and treatment.
January 2018: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/29359079/anti-n-methyl-d-aspartate-receptor-encephalitis-in-young-females
#6
Kamlesh Kumari, Neeru Sahni, Vimla Kumari, Vikas Saini
Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an immune-mediated disease commonly associated with ovarian teratoma. Anti-NMDA-receptor autoantibodies disrupt NMDA function leading to the development of psychosis, seizures and autonomic dysfunction. The treatment includes underlying tumour resection and immunosuppression. Slow recovery and unpredictable clinical course makes intensive care management of these patients challenging. We report the management of two young female patients with anti-NMDA-receptor encephalitis associated with ovarian teratoma...
December 2017: Turkish Journal of Anaesthesiology and Reanimation
https://www.readbyqxmd.com/read/29348113/genetics-in-endocrinology-the-expanding-genetic-horizon-of-primary-aldosteronism
#7
Silvia Monticone, Fabrizio Buffolo, Martina Tetti, Franco Veglio, Barbara Pasini, Paolo Mulatero
Aldosterone is the main mineralocorticoid hormone in humans and plays a key role in maintaining water and electrolyte homeostasis. Primary aldosteronism (PA), characterized by autonomous aldosterone overproduction by the adrenal glands, affects 6% of the general hypertensive population and can be either sporadic or familial. Aldosterone producing adenoma (APA) and bilateral adrenal hyperplasia (BAH) are the two most frequent subtypes of sporadic PA, and 4 forms of familial hyperaldosteronism (FH-I to FH-IV) have been identified...
January 18, 2018: European Journal of Endocrinology
https://www.readbyqxmd.com/read/29344466/comparison-of-autonomic-function-before-and-after-surgical-intervention-in-patients-with-temporal-lobe-epilepsy
#8
Navita Choudhary, Kishore K Deepak, Poodipedi S Chandra, Shalini Bhatia, Rajesh Sagar, Ashok K Jaryal, Ravindra M Pandey, Manjari Tripathi
Background and Purpose: Refractory temporal lobe epilepsy (TLE) is commonly associated with imbalances in cardiovascular (CV) parasympathetic and sympathetic functions, which are treated using TLE surgery. We investigated the effect of hemispheric lateralization of seizure foci on autonomic CV functions before and after TLE surgery. Methods: The study was conducted on patients with left TLE (LTLE, n = 23) and right TLE (RTLE, n = 30) undergoing unilateral TLE surgery...
December 2017: Journal of Epilepsy Research
https://www.readbyqxmd.com/read/29290532/thrombolysis-alert-in-hassan-ii-university-teaching-hospital-of-fez-morocco-a-prospective-study-of-2-years
#9
Moussa Toudou Daouda, Siham Bouchal, Naima Chtaou, Aouatef Midaoui, Zouahyr Souirti, Faouzi Belahsen
BACKGROUND: Thrombolysis alert (TA) is a procedure triggered every time a patient consults for sudden focal neurological deficit within 4.5 hours. OBJECTIVE: We aimed to determine firstly the etiological profile of TA and secondly to evaluate the delays in the management not only of thrombolyzed patients but also of nonthrombolyzed patients to determine the intrahospital delays to optimize. METHODS: Patients aged over 18 years who consulted for sudden focal neurological deficit within 4...
December 29, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/29289531/epileptic-seizures-are-reduced-by-autonomic-biofeedback-therapy-through-enhancement-of-fronto-limbic-connectivity-a-controlled-trial-and-neuroimaging-study
#10
Yoko Nagai, Julia Aram, Matthias Koepp, Louis Lemieux, Marco Mula, Hugo Critchley, Sanjay Sisodiya, Mara Cercignani
BACKGROUND: Thirty-percent of patients with epilepsy are drug-resistant, and might benefit from effective noninvasive therapeutic interventions. Evidence is accumulating on the efficacy of autonomic biofeedback therapy using galvanic skin response (GSR; an index of sympathetic arousal) in treating epileptic seizures. This study aimed to extend previous controlled clinical trials of autonomic biofeedback therapy with a larger homogeneous sample of patients with temporal lobe epilepsy. In addition, we used neuroimaging to characterize neural mechanisms of change in seizure frequency following the therapy...
December 13, 2017: EBioMedicine
https://www.readbyqxmd.com/read/29285879/autonomic-and-subjective-responsivity-to-emotional-images-in-people-with-dissociative-seizures
#11
Susannah Pick, John D C Mellers, Laura H Goldstein
People with dissociative seizures (DS) report a range of difficulties in emotional functioning and exhibit altered responding to emotional facial expressions in experimental tasks. We extended this research by investigating subjective and autonomic reactivity (ratings of emotional valence, arousal and skin conductance responses, and SCRs) to general emotional images in 39 people with DS relative to 42 healthy control participants, whilst controlling for anxiety, depression, cognitive functioning and, where relevant, medication use...
December 28, 2017: Journal of Neuropsychology
https://www.readbyqxmd.com/read/29282342/-motor-symptoms-of-autoimmune-encephalopathies
#12
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/29249505/early-life-epilepsies-are-a-comorbidity-of-developmental-brain-disorders
#13
Anne T Berg, Daniel Tarquinio, Sookyong Koh
Early-life epilepsies are a series of disorders frequently accompanied by a broad range of morbidities that include cognitive, behavioral, neuromuscular, and sleep disturbances; enteric and other forms of autonomic dysfunction; sensory processing difficulties; and other issues. Usually these morbidities cluster together in a single patient. Rather than these being separate conditions, all, including the seizures, are manifestations or coexpressions of developmental brain disorders. Instead of viewing epilepsy as the disease and the other features as comorbidities, approaching early-life epilepsies as part of the spectrum of developmental brain disorders could have implications for multidisciplinary care models, anticipatory guidance, and counseling of parents, as well as the design of randomized trials and targeting important outcomes...
November 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29241417/neuropsychiatric-manifestations-are-not-uncommon-in-indian-lupus-patients-and-negatively-affect-quality-of-life
#14
H Muhammed, M Goyal, V Lal, S Singh, V Dhir
Objectives The objectives of this paper are to look at the prevalence of neuropsychiatric manifestations and assess their impact on quality of life in North Indian lupus patients. Methods The study included consecutive patients with systemic lupus erythematosus (SLE) who were older than 18 years and met the SLICC 2012 criteria. A diagnosis of a neuropsychiatric syndrome was made as per ACR 1999 definitions. Manifestations occurring at any point in time after the diagnosis of SLE were considered if a reliable history and medical records were available...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/29214651/electroencephalography-and-behavior-patterns-during-experimental-status-epilepticus
#15
Ewa Lewczuk, Suchitra Joshi, John Williamson, Mouna Penmetsa, Sarah Shan, Jaideep Kapur
OBJECTIVE: To characterize the evolution of behavioral and electrographic seizures in an experimental electrical stimulation-based model of status epilepticus (SE) in C57Bl/6 mice, and to relate SE to various outcomes, including death and epileptogenesis. METHODS: SE was induced by continuous hippocampal stimulation and was evaluated by review of electroencephalographic recordings, spectral display, and behavior. RESULTS: Seizures were initially locked to the electrical trains but later became independent of them...
December 6, 2017: Epilepsia
https://www.readbyqxmd.com/read/29183618/encephalitis-associated-with-antibodies-against-the-nmda-receptor
#16
REVIEW
Mar Guasp, Josep Dalmau
The encephalitis associated with antibodies against the N-methyl-D-aspartate receptor (NMDAR) is characterized by the presence of antibodies against the GluN1 subunit of this receptor, resulting in symptoms that are similar to those observed in models of genetic or pharmacologic reduction of NMDARs. Patients are usually young adults, predominantly women, and children who develop, in a sequential manner, rapidly progressive symptoms including psychosis, abnormal movements, autonomic dysfunction, and coma. Epileptic seizures are variable and can occur throughout the course of the disease...
November 25, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/29171405/seizure-semiology-of-anti-lgi1-antibody-encephalitis
#17
Nicholas J Beimer, Linda M Selwa
Limbic encephalitis associated with anti-LGI1 antibody (LGI1 encephalitis) presents with a variety of features, the most prominent of which include seizures and progressive disturbance of memory and behaviour. Although varied in semiology, recognition of the pattern of seizures in LGI1 encephalitis is important, as early diagnosis and definitive treatment may prevent subsequent development of cognitive impairment. We present a patient with LGI1 encephalitis and "faciobrachial dystonic seizures-plus", which began as classic faciobrachial dystonic seizures and progressed to focal seizures with impaired awareness, dacrystic/gelastic-like outbursts, ictal speech, manual automatisms, and autonomic signs (tachycardia)...
December 1, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29153996/clinical-presentation-of-anti-n-methyl-d-aspartate-receptor-and-anti-voltage-gated-potassium-channel-complex-antibodies-in-children-a-series-of-24-cases
#18
Bahadir Konuskan, Mirac Yildirim, Haluk Topaloglu, Ilknur Erol, Ulkuhan Oztoprak, Huseyin Tan, Rahsan Gocmen, Banu Anlar
OBJECTIVE: The symptomatology and paraclinical findings of antibody-mediated encephalitis, a relatively novel disorder, are still being characterized in adults and children. A high index of suspicion is needed in order to identify these cases among children presenting with various neurological symptoms. The aim of this study is to examine the clinical, demographic and laboratory findings and outcome of children with anti-NMDAR and anti-VGKC encephalitis for any typical or distinctive features...
November 7, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29138603/fatal-collapse-due-to-autonomic-dysreflexia-during-manual-self-evacuation-of-bowel-in-a-tetraplegic-patient-living-alone-lessons-to-learn
#19
Subramanian Vaidyanathan, Bakul M Soni, Paul Mansour, Tun Oo
Background: To identify areas for improvement, the National Health Service in England mandates the review of case reports of patients who have died, which should be translated into improved care for other patients. Case report: A 49-year-old Caucasian man sustained C-7 tetraplegia in a motorcycle accident in 1992. In 2009, he developed seizures and collapsed in the lavatory on a number of occasions during manual self-evacuation of his bowel. A 24-hour electrocardiogram recording at that time showed sinus rhythm with a maximum heart rate of 97 and a minimum of 39 beats per minute; there were no significant arrhythmias that could have contributed to his episodes of collapse...
2017: International Medical Case Reports Journal
https://www.readbyqxmd.com/read/29116659/type-1-astrocyte-like-stem-cells-harboring-cacna1d-gene-deletion-exhibit-reduced-proliferation-and-decreased-neuronal-fate-choice
#20
Bianca Völkening, Kai Schönig, Golo Kronenberg, Dusan Bartsch, Tillmann Weber
In the central nervous system, CaV 1.2 and CaV 1. 3 constitute the main L-type voltage-gated calcium channels (LTCCs) coupling membrane depolarization to gene transcription. We have previously demonstrated that inducible disruption of Cav1.2 in type-1 astrocyte-like stem cells of the adult dentate gyrus (DG) impairs hippocampal neurogenesis in a cell-autonomous fashion. To address the role of Cav1.3 channels (encoded by the Cacna1d gene), we here generated Tg(GLAST-CreERT2) /Cacna1d(fl/fl) /RCE:loxP mice which facilitate inducible deletion of Cacna1d in tandem with induction of EGFP expression in type-1 cells, allowing tracking of recombined cells and their descendants...
November 8, 2017: Hippocampus
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