Specific characteristics and current diagnostic and treatment modalities performance of super refractory status epilepticus in children: A comparative study

2022 ◽  
Author(s):  
George Briassoulis ◽  
Chrysavgi Stefanogianni ◽  
Ioannis Zaganas ◽  
Maria Raissaki ◽  
Panagiotis Briassoulis ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Comparative Study ◽  
Refractory Status Epilepticus ◽  
Treatment Modalities ◽  
Refractory Status

scholarly journals A comparative study of midazolam and target-controlled propofol infusion in the treatment of refractory status epilepticus

2018 ◽  
Vol 22 (6) ◽  
pp. 441-448 ◽  
Author(s):  
Dheeraj Masapu ◽  
K. N. Gopala Krishna ◽  
Sinha Sanjib ◽  
Dhrithiman Chakrabarti ◽  
R. C. Mundlamuri ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Comparative Study ◽  
Refractory Status Epilepticus ◽  
Refractory 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.

scholarly journals Propofol Infusion Syndrome in Refractory Status Epilepticus: A Case Report and Topical Review

2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
Akil Walli ◽  
Troels Dirch Poulsen ◽  
Mette Dam ◽  
Jens Børglum
Keyword(s):  
Status Epilepticus ◽  
Refractory Status Epilepticus ◽  
Treatment Modalities ◽  
Fatal Complication ◽  
Propofol Infusion Syndrome ◽  
Refractory Status ◽  
Blood Exchange ◽  
New Treatment ◽  
Alternative Medications ◽  
Sedative Agents

Propofol infusion syndrome (PRIS) is a fatal complication when doses of propofol administration exceed 4 mg/kg/h for more than 48 hours. Propofol overdosage is not uncommon in patients with refractory status epilepticus (RSE). We describe a case of refractory status epilepticus complicated by propofol infusion syndrome and collect from 5 databases all reports of refractory status epilepticus cases that were treated by propofol and developed the syndrome and outline whether refractory status epilepticus treatment with propofol is standardized according to international recommendations, compare it with alternative medications, and discuss how this syndrome can be treated and prevented. A total of 21 patients who developed this syndrome reported arrhythmia in all cases (100%), rhabdomyolysis in 9 cases (42%), lactic acidosis in 13 cases (62%), renal failure in 8 cases (38%), lipemia in 7 cases (33%), and elevated hepatic enzymes in 6 cases (28%). 13 patients died (66%). Propofol is still given in a dosage higher than what is internationally recommended, and new treatment modalities such as renal replacement therapy, blood exchange, and extracorporeal membrane oxygenation seem to be promising. In conclusion, propofol should be carefully titrated, the maximal infusion rate needs to be reassessed, and combination of different sedative agents may be considered.

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.

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