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febrile infection-related epilepsy syndrome

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https://www.readbyqxmd.com/read/27780632/-symptomatic-infection-associated-acute-encephalopathy-in-children-with-underlying-neurological-disorders
#1
Yoshimichi Hirayama, Yoshiaki Saito, Yoshihiro Maegaki
BACKGROUND: Development of infection-associated acute encephalopathy (AE) is precipitated by several factors, including viral agents, age, and genetic polymorphisms. In addition, children with prior underlying neurological disorders can also present with AE. METHOD: We reviewed 55 children with AE who were referred to hospitals participating in the Status Epilepticus Study Group from 1988 to 2013. AE was classified into eight subtypes: acute encephalopathy with biphasic seizures and late reduced diffusion (AESD); hemiconvulsion-hemiplegia syndrome (HH); acute necrotizing encephalopathy; hemorrhagic shock and encephalopathy syndrome (HSES); clinically mild encephalitis/encephalopathy with a reversible splenial lesion; acute encephalitis with refractory, repetitive partial seizures; Reye-like syndrome; and unclassified...
October 22, 2016: Brain & Development
https://www.readbyqxmd.com/read/27770579/febrile-infection-related-epilepsy-syndrome-treated-with-anakinra
#2
Daniel L Kenney-Jung, Annamaria Vezzani, Robert J Kahoud, Reghann G LaFrance-Corey, Mai-Lan Ho, Theresa Wampler Muskardin, Elaine C Wirrell, Charles L Howe, Eric T Payne
Febrile infection-related epilepsy syndrome (FIRES) is a devastating epileptic encephalopathy with limited treatment options and an unclear etiology. Anakinra is a recombinant version of the human interleukin-1 receptor antagonist used to treat autoinflammatory disorders. This is the first report of anakinra for treatment of a child with super-refractory status epilepticus secondary to FIRES. Anakinra was well tolerated and effective. Cerebral spinal fluid analysis revealed elevated levels of proinflammatory cytokines before treatment that normalized on anakinra, suggesting a potential pathogenic role for neuroinflammation in FIRES...
October 22, 2016: Annals of Neurology
https://www.readbyqxmd.com/read/27538648/cytokine-related-and-sodium-channel-polymorphism-as-candidate-predisposing-factors-for-childhood-encephalopathy-fires-aerrps
#3
M Saitoh, K Kobayashi, I Ohmori, Y Tanaka, K Tanaka, T Inoue, A Horino, K Ohmura, A Kumakura, Y Takei, S Hirabayashi, M Kajimoto, T Uchida, S Yamazaki, T Shiihara, T Kumagai, M Kasai, H Terashima, M Kubota, M Mizuguchi
Febrile infection-related epilepsy syndrome (FIRES), or acute encephalitis with refractory, repetitive partial seizures (AERRPS), is an epileptic encephalopathy beginning with fever-mediated seizures. The etiology remains unclear. To elucidate the genetic background of FIRES/AERRPS (hereafter FIRES), we recruited 19 Japanese patients, genotyped polymorphisms of the IL1B, IL6, IL10, TNFA, IL1RN, SCN1A and SCN2A genes, and compared their frequency between the patients and controls. For IL1RN, the frequency of a variable number of tandem repeat (VNTR) allele, RN2, was significantly higher in the patients than in controls (p=0...
September 15, 2016: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27475280/ketogenic-diet-treatment-for-pediatric-super-refractory-status-epilepticus
#4
Brian Appavu, Lisa Vanatta, John Condie, John F Kerrigan, Randa Jarrar
PURPOSE: We aimed to study whether ketogenic diet (KD) therapy leads to resolution of super-refractory status epilepticus in pediatric patients without significant harm. METHOD: A retrospective review was performed at Phoenix Children's Hospital on patients with super-refractory status epilepticus undergoing ketogenic diet therapy from 2011 to 2015. RESULTS: Ten children with super-refractory status epilepticus, ages 2-16 years, were identified...
October 2016: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27434885/effectiveness-of-electroconvulsive-therapy-for-refractory-status-epilepticus-in-febrile-infection-related-epilepsy-syndrome
#5
Alicia Mirás Veiga, David Conejo Moreno, Ana Isabel Gómez Menéndez, Ignacio Muñoz Siscart, Marta Del Olmo Fernández, Esteban Gómez Sánchez, María García González, Fernando Gómez Sáez
Febrile infection-related epilepsy syndrome (FIRES) is a rare condition which evolves into refractory status epilepticus (SE), with poor outcome in most cases. Conventional antiepileptic drugs fail to control SE in FIRES patients. We report the case of a previously healthy 4-year-old boy who was diagnosed with FIRES. One week after pharyngitis and high fever he started seizures, followed by refractory SE. Benzodiazepines, phenytoin, high-dose barbiturates that induce burst suppression, high doses of corticosteroids, plasmapheresis, immunoglobulins, propofol, lidocaine, ketamine, inhaled desflurane, ketogenic diet, lacosamide, and therapeutic hypothermia were tried at different times in a period of 8 weeks, but all of them were ineffective...
July 19, 2016: Neuropediatrics
https://www.readbyqxmd.com/read/27358770/marked-improvement-in-febrile-infection-related-epilepsy-syndrome-after-lidocaine-plus-mgso4-treatment-in-a-12-year-old-girl
#6
I-Ching Chou, Huan-Cheng Lai, Fuu-Jen Tsai, Yu-Tzu Chang, Sheng-Shing Lin, Syuan-Yu Hong, Inn-Chi Lee
PURPOSE: This report sheds light on a successful treatment in febrile infection-related epilepsy syndrome (FIRES) with the combined use of lidocaine and MgSO4. METHODS: We report a 12-year-old previously healthy girl who experienced an upper respiratory infection with fever and headache for 2 days, then suddenly went into a coma followed by repetitive status epilepticus. All tests for CNS infection, metabolic and toxic diseases, and autoimmune encephalitis were negative...
2016: Epilepsy & Behavior Case Reports
https://www.readbyqxmd.com/read/27293328/clinical-profile-and-treatment-outcome-of-febrile-infection-related-epilepsy-syndrome-in-south-indian-children
#7
Sandeep B Patil, Arun Grace Roy, Kollencheri Puthenveettil Vinayan
PURPOSE: To describe the clinical features and outcome of febrile infection-related epilepsy syndrome (FIRES), a catastrophic epileptic encephalopathy, in a cohort of South Indian children. MATERIALS AND METHODS: We performed a retrospective chart review of a cohort of children with previously normal development who presented with status epilepticus or encephalopathy with recurrent seizures following a nonspecific febrile illness during the period between January 2007 and January 2012...
April 2016: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/27000515/a-systematic-review-of-the-risks-factors-associated-with-the-onset-and-natural-progression-of-epilepsy
#8
Stephanie Walsh, Jennifer Donnan, Yannick Fortin, Lindsey Sikora, Andrea Morrissey, Kayla Collins, Don MacDonald
Epilepsy is a neurological condition that affects more than 50 million individuals worldwide. It presents as unpredictable, temporary and recurrent seizures often having negative physical, psychological and social consequences. To inform disease prevention and management strategies, a comprehensive systematic review of the literature on risk factors for the onset and natural progression of epilepsy was conducted. Computerized bibliographic databases for systematic reviews, meta-analyses, observational studies and genetic association studies published between 1990 and 2013 describing etiological risk factors for epilepsy was searched...
March 18, 2016: Neurotoxicology
https://www.readbyqxmd.com/read/26597039/chronological-evolution-of-magnetic-resonance-imaging-findings-in-children-with-febrile-infection-related-epilepsy-syndrome
#9
Marianna S Rivas-Coppola, Namrata Shah, Asim F Choudhri, Robin Morgan, James W Wheless
OBJECTIVE: To describe and analyze the chronological evolution of the radiological findings in seven children with febrile infection-related epilepsy syndrome. METHODS: This is a retrospective study describing the radiological findings and evolution in seven children with febrile infection-related epilepsy syndrome who presented from 2009 to 2013. The children all fit the defined clinical criteria for febrile infection-related epilepsy syndrome; all had a history of normal psychomotor development who presented with acute-onset catastrophic partial status epilepticus associated with a febrile illness or unspecific infectious process...
February 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/26379807/hippocampal-changes-in-febrile-infection-related-epilepsy-syndrome-fires
#10
Amit Agarwal, Shyamsunder Sabat, Krishnamurthy Thamburaj, Sangam Kanekar
BACKGROUND: Febrile seizures are the most common seizure disorder in childhood, associated with a significant rise in body temperature. However, post-infectious refractory afebrile form of seizures in previously healthy children is being increasingly recognized in around the world, which evolves into a chronic refractory form of epilepsy. The term 'Febrile infection-related epilepsy syndrome' (FIRES) has been proposed for these conditions and represents a refractory severe post-infectious epileptic condition in previously normal children...
2015: Polish Journal of Radiology
https://www.readbyqxmd.com/read/26060905/rasmussen-syndrome-and-other-inflammatory-epilepsies
#11
REVIEW
Sophia Varadkar, J Helen Cross
An underlying immune basis is emerging in an increasing number of epileptic and encephalopathic syndromes. The immunopathological mechanisms may be categorized into antibody-mediated, T-cell cytotoxicity, and microglia-induced degeneration. The immune basis in Rasmussen syndrome is thought to be T-cell mediated. Antibodies to extracellular and intracellular epitopes are implicated in limbic and other encephalitides, characterized by seizures, movement disorder, sleep disorder, obtundation, psychosis, mutism, and other psychiatric symptoms...
June 2015: Seminars in Neurology
https://www.readbyqxmd.com/read/25917466/febrile-seizures-and-genetic-epilepsy-with-febrile-seizures-plus-gefs
#12
REVIEW
Peter Camfield, Carol Camfield
To review the literature about febrile seizures and GEFS plus with special emphasis on management and outcome. Selected literature review. Febrile seizures are the most common convulsive event in humans, occurring in 2-6% of the population. The aetiology is complex with strong evidence for a heterogeneous genetic predisposition interacting with fever of any cause, with certain viral infections having a greater effect. A large amount of literature has established that febrile seizures have no long-term consequences on cognition or behaviour...
June 2015: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/25727510/evaluation-and-treatment-of-autoimmune-neurologic-disorders-in-the-pediatric-intensive-care-unit
#13
REVIEW
Leslie A Benson, Heather Olson, Mark P Gorman
Autoimmunity is being increasingly recognized as a cause of neurologic presentations both inside and outside the intensive care unit (ICU) setting. Pediatric autoimmune neurologic diseases likely to be seen in the ICU include autoimmune encephalitidies such as N-Methyl-D-aspartate (NMDA) receptor encephalitis, central nervous system vasculitis, demyelinating disorders, and neurologic involvement of systemic autoimmune disorders. In addition, there are conditions of suspected autoimmune etiology such as febrile infection-related epilepsy syndrome (FIRES) and rapid-onset obesity, hypoventilation, hypothalamic dysfunction, and autonomic dysregulation (ROHHAD) syndrome that are rare, but when they do present, it is often to the ICU...
December 2014: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/25332495/cognitive-outcomes-in-febrile-infection-related-epilepsy-syndrome-treated-with-the-ketogenic-diet
#14
Rani K Singh, Sucheta M Joshi, Denise M Potter, Steve M Leber, Martha D Carlson, Renée A Shellhaas
Febrile infection-related epilepsy syndrome (FIRES) is a newly recognized epileptic encephalopathy in which previously healthy school-aged children present with prolonged treatment-resistant status epilepticus (SE). Survivors are typically left with pharmacoresistant epilepsy and severe cognitive impairment. Various treatment regimens have been reported, all with limited success. The ketogenic diet (KD) is an alternative treatment of epilepsy and may be an appropriate choice for children with refractory SE...
November 2014: Pediatrics
https://www.readbyqxmd.com/read/25015494/human-herpesvirus-6-and-the-nervous-system
#15
REVIEW
Joshua A Hill, Nagagopal Venna
No abstract text is available yet for this article.
2014: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/24927446/-febrile-infection-related-epilepsy-syndrome-in-two-children
#16
Hong-Ling Wei, Ling Wei, Ya-Nan Tang
No abstract text is available yet for this article.
June 2014: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/24639375/mechanisms-of-epileptogenesis-in-pediatric-epileptic-syndromes-rasmussen-encephalitis-infantile-spasms-and-febrile-infection-related-epilepsy-syndrome-fires
#17
REVIEW
Carlos A Pardo, Rima Nabbout, Aristea S Galanopoulou
The mechanisms of epileptogenesis in pediatric epileptic syndromes are diverse, and may involve disturbances of neurodevelopmental trajectories, synaptic homeostasis, and cortical connectivity, which may occur during brain development, early infancy, or childhood. Although genetic or structural/metabolic factors are frequently associated with age-specific epileptic syndromes, such as infantile spasms and West syndrome, other syndromes may be determined by the effect of immunopathogenic mechanisms or energy-dependent processes in response to environmental challenges, such as infections or fever in normally-developed children during early or late childhood...
April 2014: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/24477659/actual-insights-into-the-clinical-management-of-febrile-seizures
#18
REVIEW
Mario Mastrangelo, Fabio Midulla, Corrado Moretti
UNLABELLED: Febrile seizures (FS) are a benign epileptic manifestation of infancy occurring between 3 months and 5 years of age and affecting an estimated 2-5 % of children. They have usually no important negative effects on motor and cognitive development. Simple FS (generalized seizures, lasting less than 10 min and single episodes during the same febrile event) have a benign prognosis in almost all cases and do not require an extensive diagnostic workup. In complex FS (focal semiology and lasting more than 10 min, more than one episode during the same febrile event), a more detailed clinical, electroencephalographic, laboratory, and neuroimaging evaluation is necessary because of a higher percentage of underlying detectable causes and a mildly higher risk for later development of epilepsy...
August 2014: European Journal of Pediatrics
https://www.readbyqxmd.com/read/24001074/fires-and-ihhe-delineation-of-the-syndromes
#19
REVIEW
Rima Nabbout
Idiopathic hemiconvulsion hemiplegia and epilepsy syndrome (IHHE) and febrile infection-related epilepsy syndrome (FIRES) are rare epileptic syndromes characterized by the occurrence of status epilepticus in a previously healthy child during or closely after a febrile episode. In both syndromes, there is no evidence of central nervous system infection (encephalitis) and the etiology remains unclear. Treatment is disappointing, particularly in FIRES, except for a response to ketogenic diet (KD) in half of patients...
September 2013: Epilepsia
https://www.readbyqxmd.com/read/23794447/reversible-splenic-lesion-in-a-patient-with-febrile-infection-related-epilepsy-syndrome-fires
#20
Fumihito Nozaki, Tomohiro Kumada, Tomoko Miyajima, Takashi Kusunoki, Ikuko Hiejima, Anri Hayashi, Tatsuya Fujii
Febrile infection-related epilepsy syndrome (FIRES) is a severe epileptic syndrome that manifests with refractory seizures or status epilepticus in previously healthy children after banal febrile illness. The neuroimaging findings in the acute phase of FIRES are nonspecific or normal. We report the case of a 7-year-old boy with FIRES who presented with a reversible lesion in the splenium of the corpus callosum on brain magnetic resonance imaging (MRI). The patient developed clusters of clonic seizures with a deviation of the eyes after a 3-day history of fever...
October 2013: Neuropediatrics
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