scholarly journals Treatment of Super-Refractory Status Epilepticus: A Review

2021 ◽  
pp. 153575972199967
Author(s):  
Juan G. Ochoa ◽  
Michelle Dougherty ◽  
Alex Papanastassiou ◽  
Barry Gidal ◽  
Ismail Mohamed ◽  
...  

Purpose: Super-refractory status epilepticus (SRSE) presents management challenges due to the absence of randomized controlled trials and a plethora of potential medical therapies. The literature on treatment options for SRSE reports variable success and quality of evidence. This review is a sequel to the 2020 American Epilepsy Society (AES) comprehensive review of the treatment of convulsive refractory status epilepticus (RSE). Methods: We sought to determine the effectiveness of treatment options for SRSE. We performed a structured literature search (MEDLINE, Embase, CENTRAL, CINAHL) for studies on reported treatments of SRSE. We excluded antiseizure medications (ASMs) covered in the 2016 AES guideline on the treatment of established SE and the convulsive RSE comprehensive review of the 2020 AES. Literature was reviewed on the effectiveness of vagus nerve stimulation, ketogenic diet (KD), lidocaine, inhalation anesthetics, brain surgery, therapeutic hypothermia, perampanel, pregabalin (PGB), and topiramate in the treatment of SRSE. Two authors reviewed each therapeutic intervention. We graded the level of the evidence according to the 2017 classification scheme of the American Academy of Neurology. Results: For SRSE (level U; 39 class IV studies total), insufficient evidence exists to support that perampanel, PGB, lidocaine, or acute vagus nerve stimulation (VNS) is effective. For children and adults with SRSE, insufficient evidence exists to support that the KD is effective (level U; 5 class IV studies). For adults with SRSE, insufficient evidence exists that brain surgery is effective (level U, 7 class IV studies). For adults with SRSE insufficient, evidence exists that therapeutic hypothermia is effective (level C, 1 class II and 4 class IV studies). For neonates with hypoxic-ischemic encephalopathy, insufficient evidence exists that therapeutic hypothermia reduces seizure burden (level U; 1 class IV study). For adults with SRSE, insufficient evidence exists that inhalation anesthetics are effective (level U, 1 class IV study) and that there is a potential risk of neurotoxicity. Conclusion: For patients with SRSE insufficient, evidence exists that any of the ASMs reviewed, inhalational anesthetics, ketogenic diet, acute VNS, brain surgery, and therapeutic hypothermia are effective treatments. Data supporting the use of these treatments for SRSE are scarce and limited mainly to small case series and case reports and are confounded by differences in patients’ population, and comedications, among other factors.

2020 ◽  
Vol 20 (5) ◽  
pp. 245-264
Author(s):  
David G. Vossler ◽  
Jacquelyn L. Bainbridge ◽  
Jane G. Boggs ◽  
Edward J. Novotny ◽  
Tobias Loddenkemper ◽  
...  

Purpose: Established tonic–clonic status epilepticus (SE) does not stop in one-third of patients when treated with an intravenous (IV) benzodiazepine bolus followed by a loading dose of a second antiseizure medication (ASM). These patients have refractory status epilepticus (RSE) and a high risk of morbidity and death. For patients with convulsive refractory status epilepticus (CRSE), we sought to determine the strength of evidence for 8 parenteral ASMs used as third-line treatment in stopping clinical CRSE. Methods: A structured literature search (MEDLINE, Embase, CENTRAL, CINAHL) was performed to identify original studies on the treatment of CRSE in children and adults using IV brivaracetam, ketamine, lacosamide, levetiracetam (LEV), midazolam (MDZ), pentobarbital (PTB; and thiopental), propofol (PRO), and valproic acid (VPA). Adrenocorticotropic hormone (ACTH), corticosteroids, intravenous immunoglobulin (IVIg), magnesium sulfate, and pyridoxine were added to determine the effectiveness in treating hard-to-control seizures in special circumstances. Studies were evaluated by predefined criteria and were classified by strength of evidence in stopping clinical CRSE (either as the last ASM added or compared to another ASM) according to the 2017 American Academy of Neurology process. Results: No studies exist on the use of ACTH, corticosteroids, or IVIg for the treatment of CRSE. Small series and case reports exist on the use of these agents in the treatment of RSE of suspected immune etiology, severe epileptic encephalopathies, and rare epilepsy syndromes. For adults with CRSE, insufficient evidence exists on the effectiveness of brivaracetam (level U; 4 class IV studies). For children and adults with CRSE, insufficient evidence exists on the effectiveness of ketamine (level U; 25 class IV studies). For children and adults with CRSE, it is possible that lacosamide is effective at stopping RSE (level C; 2 class III, 14 class IV studies). For children with CRSE, insufficient evidence exists that LEV and VPA are equally effective (level U, 1 class III study). For adults with CRSE, insufficient evidence exists to support the effectiveness of LEV (level U; 2 class IV studies). Magnesium sulfate may be effective in the treatment of eclampsia, but there are only case reports of its use for CRSE. For children with CRSE, insufficient evidence exists to support either that MDZ and diazepam infusions are equally effective (level U; 1 class III study) or that MDZ infusion and PTB are equally effective (level U; 1 class III study). For adults with CRSE, insufficient evidence exists to support either that MDZ infusion and PRO are equally effective (level U; 1 class III study) or that low-dose and high-dose MDZ infusions are equally effective (level U; 1 class III study). For children and adults with CRSE, insufficient evidence exists to support that MDZ is effective as the last drug added (level U; 29 class IV studies). For adults with CRSE, insufficient evidence exists to support that PTB and PRO are equally effective (level U; 1 class III study). For adults and children with CRSE, insufficient evidence exists to support that PTB is effective as the last ASM added (level U; 42 class IV studies). For CRSE, insufficient evidence exists to support that PRO is effective as the last ASM used (level U; 26 class IV studies). No pediatric-only studies exist on the use of PRO for CRSE, and many guidelines do not recommend its use in children aged <16 years. Pyridoxine-dependent and pyridoxine-responsive epilepsies should be considered in children presenting between birth and age 3 years with refractory seizures and no imaging lesion or other acquired cause of seizures. For children with CRSE, insufficient evidence exists that VPA and diazepam infusion are equally effective (level U, 1 class III study). No class I to III studies have been reported in adults treated with VPA for CRSE. In comparison, for children and adults with established convulsive SE (ie, not RSE), after an initial benzodiazepine, it is likely that loading doses of LEV 60 mg/kg, VPA 40 mg/kg, and fosphenytoin 20 mg PE/kg are equally effective at stopping SE (level B, 1 class I study). Conclusions: Mostly insufficient evidence exists on the efficacy of stopping clinical CRSE using brivaracetam, lacosamide, LEV, valproate, ketamine, MDZ, PTB, and PRO either as the last ASM or compared to others of these drugs. Adrenocorticotropic hormone, IVIg, corticosteroids, magnesium sulfate, and pyridoxine have been used in special situations but have not been studied for CRSE. For the treatment of established convulsive SE (ie, not RSE), LEV, VPA, and fosphenytoin are likely equally effective, but whether this is also true for CRSE is unknown. Triple-masked, randomized controlled trials are needed to compare the effectiveness of parenteral anesthetizing and nonanesthetizing ASMs in the treatment of CRSE.


2020 ◽  
Vol 43 (3) ◽  
pp. 277-284 ◽  
Author(s):  
Mei-Hsin Hsu ◽  
Hsuan-Chang Kuo ◽  
Jainn-Jim Lin ◽  
Ming-Yi Chou ◽  
Ying-Jui Lin ◽  
...  

2015 ◽  
Vol 5 (4) ◽  
pp. 198-202 ◽  
Author(s):  
Sandra D.W. Buttram ◽  
Alicia K. Au ◽  
Joshua Koch ◽  
Karen Lidsky ◽  
Kristin McBain ◽  
...  

Author(s):  
Frederick A. Zeiler ◽  
Kaitlin J. Zeiler ◽  
Jeanne Teitelbaum ◽  
Lawrence M. Gillman ◽  
Michael West

AbstractBackground: Our goal was to perform a systematic review of the literature on the use of therapeutic hypothermia for refractory status epilepticus (RSE) and its impact on seizure control. Methods: All articles from MEDLINE, BIOSIS, EMBASE, Global Health, HealthStar, Scopus, Cochrane Library, the International Clinical Trials Registry Platform (inception to May 2014), reference lists of relevant articles, and gray literature were searched. The strength of evidence was adjudicated by two independent reviewers, using both the Oxford and GRADE methodology. Results: Overall, 13 studies were identified, with 10 manuscripts and 3 meeting abstracts. A total of 40 patients were treated. The common target temperature was 33 degrees Celsius, sustained for a median 48 hours. Patients displayed a 62.5%, 15% and 22.5% rate of seizure cessation, seizure reduction, and failure of treatment respectively. External cooling was utilized in the majority of cases. Deep venous thrombosis, coagulopathy and infections were the commonly reported complications. Two ongoing clinical trials were identified. Conclusions: Oxford level 4, GRADE D evidence exists to support the use of therapeutic hypothermia to control seizures in RSE. Further prospective study is warranted.


2018 ◽  
Vol 2018 ◽  
pp. 1-19 ◽  
Author(s):  
Rohit Marawar ◽  
Maysaa Basha ◽  
Advait Mahulikar ◽  
Aaron Desai ◽  
Kushak Suchdev ◽  
...  

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.


Author(s):  
Remya Reghu ◽  
Aloysius James ◽  
Reshma Reji ◽  
Bini Vincent ◽  
Nimmu Mani

ABSTRACTFebrile infection-related epilepsy syndrome is characterized by super refractory status epilepticus that is resistant to conventional antiepileptic drugs.This case report critically analyses the treatment options adopted in a hospital to manage this syndrome. Despite the aggressive efforts that weretaken, the patient succumbed to the complications of the illness and side effects of the treatment strategies adopted. This shows that the treatmentoptions currently available are in adequate, so an intensive research in the area of pathogenesis of status epilepticus is required to frame treatmentstrategies that can bring out better outcomes.Keywords: Antiepileptics, Febrile infection-related epilepsy syndrome, Status epilepticus.


Sign in / Sign up

Export Citation Format

Share Document