Febrile Infection-Related Epilepsy Syndrome

2019 ◽  
Vol 08 (03) ◽  
pp. 083-092
Author(s):  
Mandeep Rana ◽  
Alcy R. Torres ◽  
Kam Lun Hon ◽  
Alexander K.C. Leung ◽  
Rinat Jonas
Keyword(s):  
Status Epilepticus ◽  
Refractory Status Epilepticus ◽  
Febrile Episode ◽  
Epilepsy Syndrome ◽  
Future Directions ◽  
Refractory Status ◽  
New Treatment ◽  
A Minor ◽  
Autoimmune Etiology ◽  
New Onset

AbstractFebrile infection-related epilepsy syndrome (FIRES) is a subset or variant of new-onset refractory status epilepticus in children. FIRES is characterized by the occurrence of a febrile episode between 24 hours and 2 weeks before the onset of refractory status epilepticus. A infectious cause is rarely identified in FIRES and an inflammatory or autoimmune etiology is implied. Seizures in FIRES are very difficult to control, and treatments include antiepileptic drugs, ketogenic diet, intravenous immunoglobulin, plasmapheresis, and corticosteroid therapy. The prognosis for patients with FIRES is poor, and most children are left with refractory epilepsy and cognitive impairment. The new consensus guidelines on the terminology of FIRES and recent interest in new treatment approaches have been welcome developments for clinicians who face the challenge of diagnosing and managing status epilepticus in a previously healthy child that occurs following a minor febrile episode. This review aims to provide clinicians with an update on the current hypotheses for the etiology, pathogenesis, clinical evaluation, management, and future directions in the diagnosis and treatment of FIRES.

scholarly journals How to Help Your Patients Enroll in the New-Onset Refractory Status Epilepticus (NORSE) and Febrile Infection-Related Epilepsy Syndrome (FIRES) Family Registry, and Other Rare Epilepsy Registries

2021 ◽  
pp. 153575972199832
Author(s):  
Karnig Kazazian ◽  
Marissa Kellogg ◽  
Nora Wong ◽  
Krista Eschbach ◽  
Raquel Farias Moeller ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Refractory Status Epilepticus ◽  
Epilepsy Syndrome ◽  
Neurologic Disorder ◽  
Mortality And Morbidity ◽  
Refractory Status ◽  
Rigorous Research ◽  
Active Epilepsy ◽  
New Onset

New-onset refractory status epilepticus (NORSE) is a rare clinical presentation of refractory status epilepticus (RSE) that occurs in people without active epilepsy or preexisting neurologic disorder. Febrile infection-related epilepsy syndrome (FIRES) is a subcategory of NORSE. New-onset refractory status epilepticus/FIRES are becoming increasingly recognized; however, information pertaining to disease course, clinical outcomes, and survivorship remains limited, and mortality and morbidity are variable, but often high. The objective of the NORSE/FIRES Family Registry is to (1) provide an easily accessible and internationally available multilingual registry into which survivors or NORSE/FIRES surrogates or family members of people affected by NORSE/FIRES or their physicians can enter data in a systematic and rigorous research study from anywhere in the world where internet is available; and (2) to examine past medical history, outcomes, and quality of life for people affected by NORSE/FIRES.

New onset refractory status epilepticus research

Neurology ◽  
2019 ◽  
Vol 92 (17) ◽  
pp. 802-810 ◽  
Author(s):  
Teneille E. Gofton ◽  
Nicolas Gaspard ◽  
Sara E. Hocker ◽  
Tobias Loddenkemper ◽  
Lawrence J. Hirsch
Keyword(s):  
Status Epilepticus ◽  
Professional Education ◽  
Age Groups ◽  
Refractory Status Epilepticus ◽  
Epilepsy Syndrome ◽  
Current State ◽  
Refractory Status ◽  
Management Approaches ◽  
Active Epilepsy ◽  
New Onset

New-onset refractory status epilepticus (NORSE) is a clinical presentation, not a specific diagnosis, in a patient without active epilepsy or other preexisting relevant neurologic disorder, with new onset of refractory status epilepticus (RSE) that does not resolve after 2 or more rescue medications, without a clear acute or active structural, toxic, or metabolic cause. Febrile infection-related epilepsy syndrome is a subset of NORSE in which fever began at least 24 hours prior to the RSE. Both terms apply to all age groups. Until recently, NORSE was a poorly recognized entity without a consistent definition or approach to care. We review the current state of knowledge in NORSE and propose a roadmap for future collaborative research. Research investigating NORSE should prioritize the following 4 domains: (1) clinical features, etiology, and pathophysiology; (2) treatment; (3) adult and pediatric evaluation and management approaches; and (4) public advocacy, professional education, and family support. We consider international collaboration and multicenter research crucial in achieving these goals.

New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives

Epilepsia ◽  
2018 ◽  
Vol 59 (4) ◽  
pp. 745-752 ◽  
Author(s):  
Nicolas Gaspard ◽  
Lawrence J. Hirsch ◽  
Claudine Sculier ◽  
Tobias Loddenkemper ◽  
Andreas van Baalen ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
State Of The Art ◽  
Refractory Status Epilepticus ◽  
Epilepsy Syndrome ◽  
Refractory Status ◽  
New Onset

scholarly journals Infantile hemiconvulsion-hemiplegia epilepsy syndrome associated with GRIN2A gene mutation

2021 ◽  
pp. 49-49
Author(s):  
Ruzica Kravljanac ◽  
Slobodan Gazikalovic ◽  
Biljana Vucetic-Tadic
Keyword(s):  
Status Epilepticus ◽  
Right Hemisphere ◽  
Refractory Status Epilepticus ◽  
Epilepsy Syndrome ◽  
Refractory Status ◽  
Specific Syndrome ◽  
First Time ◽  
Infectious Encephalitis ◽  
New Onset

Introduction. Infantile hemiconvulsion-hemiplegia and epilepsy (IHHE) syndrome is defined as a specific syndrome in a patient < 2 years of age, presenting as a new onset refractory status epilepticus with unilateral motor seizures and acute imaging abnormalities, fever, hemiparesis >24 hours, and excluding infectious encephalitis. Case outline. We present results of follow-up in 11 year-old girl with IHHE, associated with GRIN2A mutation. The girl had normal development until the first febrile hemiconvulsive status epilepticus at the age of seven months. Neuroimaging initially showed right hemisphere edema, followed by progressive right side hemiatrophy. The patient has resistant epilepsy, left side hemiparesis, and good language and cognitive development. Conclusion. Despite IHHE described many years ago, some syndrome's features, including etiology have remained unexplained. The association of IHHE and GRIN2A mutation is described in current manuscript for the first time in scientific literature.

scholarly journals A case of new-onset refractory status epilepticus (NORSE) with an autoimmune etiology

2015 ◽  
Vol 55 (12) ◽  
pp. 909-913 ◽  
Author(s):  
Teruyuki Ishikura ◽  
Tatsusada Okuno ◽  
Katsuya Araki ◽  
Masanori P. Takahashi ◽  
Kenji Watabe ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Refractory Status Epilepticus ◽  
Refractory Status ◽  
Autoimmune Etiology ◽  
New Onset

scholarly journals Updates in Refractory Status Epilepticus

2018 ◽  
Vol 2018 ◽  
pp. 1-19 ◽  
Author(s):  
Rohit Marawar ◽  
Maysaa Basha ◽  
Advait Mahulikar ◽  
Aaron Desai ◽  
Kushak Suchdev ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Treatment Options ◽  
Neurological Status ◽  
Refractory Status Epilepticus ◽  
Focal Lesion ◽  
Long Term Outcomes ◽  
Refractory Status ◽  
Autoimmune Etiology ◽  
New Onset

Refractory status epilepticus is defined as persistent seizures despite appropriate use of two intravenous medications, one of which is a benzodiazepine. It can be seen in up to 40% of cases of status epilepticus with an acute symptomatic etiology as the most likely cause. New-onset refractory status epilepticus (NORSE) is a recently coined term for refractory status epilepticus where no apparent cause is found after initial testing. A large proportion of NORSE cases are eventually found to have an autoimmune etiology needing immunomodulatory treatment. Management of refractory status epilepticus involves treatment of an underlying etiology in addition to intravenous anesthetics and antiepileptic drugs. Alternative treatment options including diet therapies, electroconvulsive therapy, and surgical resection in case of a focal lesion should be considered. Short-term and long-term outcomes tend to be poor with significant morbidity and mortality with only one-third of patients reaching baseline neurological status.

scholarly journals New-onset refractory status epilepticus (NORSE)

2021 ◽  
Vol 21 (2) ◽  
pp. 119-127
Author(s):  
Laura Mantoan Ritter ◽  
Lina Nashef
Keyword(s):  
Status Epilepticus ◽  
Immune Modulation ◽  
Functional Disability ◽  
Significant Risk ◽  
Refractory Status Epilepticus ◽  
Treatment Modalities ◽  
Epilepsy Syndrome ◽  
Refractory Status ◽  
Interleukin Inhibitors ◽  
New Onset

New-onset refractory status epilepticus and its subcategory febrile infection-related epilepsy syndrome are rare devastating clinical presentations in those without pre-existing relevant history, often in schoolchildren or young adults, without a clear cause on initial investigations. A cause is later identified in up to half of adults, but in many fewer children. Patients often require protracted intensive care and are at significant risk of dying. Functional disability is common and subsequent chronic epilepsy is the norm, but some people do have good outcomes, even after prolonged status epilepticus. Patients need prompt investigations and treatment. Anaesthetic and antiseizure medications are supplemented by other treatment modalities, including the ketogenic diet. Despite limited evidence, it is appropriate to try to modify the presumed underlying pathogenesis with immune modulation early, with a more recent focus on using interleukin inhibitors. Optimising management will require concerted multicentre international efforts.

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