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Super-refractory status epilepticus

MedGen UID:
1708961
•Concept ID:
C5397873
•
Disease or Syndrome
HPO: HP:0032868

Definition

Super-refractory status epilepticus is defined as refractory status epilepticus continuing for 24 h or more following initiation of anesthetic medications, including cases in which seizure control is attained after induction of anesthetic drugs but recurs on weaning the patient off the anesthetic agent. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSuper-refractory status epilepticus

Professional guidelines

PubMed

Management of Status Epilepticus, Refractory Status Epilepticus, and Super-refractory Status Epilepticus.
Trinka E, Leitinger M
Continuum (Minneap Minn) 2022 Apr 1;28(2):559-602. doi: 10.1212/CON.0000000000001103. PMID: 35393970
Neurostimulation in the treatment of refractory and super-refractory status epilepticus.
Trinka E, Brigo F
Epilepsy Behav 2019 Dec;101(Pt B):106551. Epub 2019 Oct 30 doi: 10.1016/j.yebeh.2019.106551. PMID: 31676239
Treatment of Refractory and Super-refractory Status Epilepticus.
Rai S, Drislane FW
Neurotherapeutics 2018 Jul;15(3):697-712. doi: 10.1007/s13311-018-0640-5. PMID: 29922905Free PMC Article

Recent clinical studies

Etiology

Neuromodulation in Super-refractory Status Epilepticus.
Stavropoulos I, Pak HL, Valentin A
J Clin Neurophysiol 2021 Nov 1;38(6):494-502. doi: 10.1097/WNP.0000000000000710. PMID: 34261110
Ketamine to treat super-refractory status epilepticus.
Alkhachroum A, Der-Nigoghossian CA, Mathews E, Massad N, Letchinger R, Doyle K, Chiu WT, Kromm J, Rubinos C, Velazquez A, Roh D, Agarwal S, Park S, Connolly ES, Claassen J
Neurology 2020 Oct 20;95(16):e2286-e2294. Epub 2020 Sep 1 doi: 10.1212/WNL.0000000000010611. PMID: 32873691Free PMC Article
Refractory and Super-refractory Status Epilepticus.
Samanta D, Garrity L, Arya R
Indian Pediatr 2020 Mar 15;57(3):239-253. doi: 10.1007/s13312-020-1759-0. PMID: 32198865Free PMC Article
Treatment of Refractory and Super-refractory Status Epilepticus.
Rai S, Drislane FW
Neurotherapeutics 2018 Jul;15(3):697-712. doi: 10.1007/s13311-018-0640-5. PMID: 29922905Free PMC Article
Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions.
Hirsch LJ, Gaspard N, van Baalen A, Nabbout R, Demeret S, Loddenkemper T, Navarro V, Specchio N, Lagae L, Rossetti AO, Hocker S, Gofton TE, Abend NS, Gilmore EJ, Hahn C, Khosravani H, Rosenow F, Trinka E
Epilepsia 2018 Apr;59(4):739-744. Epub 2018 Feb 5 doi: 10.1111/epi.14016. PMID: 29399791

Diagnosis

Management of Status Epilepticus, Refractory Status Epilepticus, and Super-refractory Status Epilepticus.
Trinka E, Leitinger M
Continuum (Minneap Minn) 2022 Apr 1;28(2):559-602. doi: 10.1212/CON.0000000000001103. PMID: 35393970
Refractory and Super-refractory Status Epilepticus.
Samanta D, Garrity L, Arya R
Indian Pediatr 2020 Mar 15;57(3):239-253. doi: 10.1007/s13312-020-1759-0. PMID: 32198865Free PMC Article
Pediatric refractory and super-refractory status epilepticus.
Vasquez A, Farias-Moeller R, Tatum W
Seizure 2019 May;68:62-71. Epub 2018 May 19 doi: 10.1016/j.seizure.2018.05.012. PMID: 29941225
Treatment of Refractory and Super-refractory Status Epilepticus.
Rai S, Drislane FW
Neurotherapeutics 2018 Jul;15(3):697-712. doi: 10.1007/s13311-018-0640-5. PMID: 29922905Free PMC Article
Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions.
Hirsch LJ, Gaspard N, van Baalen A, Nabbout R, Demeret S, Loddenkemper T, Navarro V, Specchio N, Lagae L, Rossetti AO, Hocker S, Gofton TE, Abend NS, Gilmore EJ, Hahn C, Khosravani H, Rosenow F, Trinka E
Epilepsia 2018 Apr;59(4):739-744. Epub 2018 Feb 5 doi: 10.1111/epi.14016. PMID: 29399791

Therapy

Why ketamine.
Coles L, Rosenthal ES, Bleck TP, Elm J, Zehtabchi S, Chamberlain J, Cloyd J, Shinnar S, Silbergleit R, Kapur J
Epilepsy Behav 2023 Apr;141:109066. Epub 2023 Jan 4 doi: 10.1016/j.yebeh.2022.109066. PMID: 36609129Free PMC Article
Ketamine to treat super-refractory status epilepticus.
Alkhachroum A, Der-Nigoghossian CA, Mathews E, Massad N, Letchinger R, Doyle K, Chiu WT, Kromm J, Rubinos C, Velazquez A, Roh D, Agarwal S, Park S, Connolly ES, Claassen J
Neurology 2020 Oct 20;95(16):e2286-e2294. Epub 2020 Sep 1 doi: 10.1212/WNL.0000000000010611. PMID: 32873691Free PMC Article
Refractory and Super-refractory Status Epilepticus.
Samanta D, Garrity L, Arya R
Indian Pediatr 2020 Mar 15;57(3):239-253. doi: 10.1007/s13312-020-1759-0. PMID: 32198865Free PMC Article
Pediatric refractory and super-refractory status epilepticus.
Vasquez A, Farias-Moeller R, Tatum W
Seizure 2019 May;68:62-71. Epub 2018 May 19 doi: 10.1016/j.seizure.2018.05.012. PMID: 29941225
Status Epilepticus, Refractory Status Epilepticus, and Super-refractory Status Epilepticus.
Nelson SE, Varelas PN
Continuum (Minneap Minn) 2018 Dec;24(6):1683-1707. doi: 10.1212/CON.0000000000000668. PMID: 30516601

Prognosis

Phenobarbital in super-refractory status epilepticus (PIRATE): A retrospective, multicenter analysis.
Kunst S, Rojo M, Schmidbauer ML, Pelz JO, Mueller A, Minnerup J, Meyer L, Madžar D, Reindl C, Madlener M, Malter M, Neumann B, Dimitriadis K; IGNITE Study Group
Epilepsia 2023 Jun;64(6):1482-1492. Epub 2023 Apr 16 doi: 10.1111/epi.17608. PMID: 37021609
Predicting the progression to super-refractory status epilepticus: A machine-learning study.
Brigo F, Turcato G, Lattanzi S, Orlandi N, Turchi G, Zaboli A, Giovannini G, Meletti S
J Neurol Sci 2022 Dec 15;443:120481. Epub 2022 Oct 28 doi: 10.1016/j.jns.2022.120481. PMID: 36332322
Management of Status Epilepticus, Refractory Status Epilepticus, and Super-refractory Status Epilepticus.
Trinka E, Leitinger M
Continuum (Minneap Minn) 2022 Apr 1;28(2):559-602. doi: 10.1212/CON.0000000000001103. PMID: 35393970
Status Epilepticus, Refractory Status Epilepticus, and Super-refractory Status Epilepticus.
Nelson SE, Varelas PN
Continuum (Minneap Minn) 2018 Dec;24(6):1683-1707. doi: 10.1212/CON.0000000000000668. PMID: 30516601
Treatment of Refractory and Super-refractory Status Epilepticus.
Rai S, Drislane FW
Neurotherapeutics 2018 Jul;15(3):697-712. doi: 10.1007/s13311-018-0640-5. PMID: 29922905Free PMC Article

Clinical prediction guides

Phenobarbital in super-refractory status epilepticus (PIRATE): A retrospective, multicenter analysis.
Kunst S, Rojo M, Schmidbauer ML, Pelz JO, Mueller A, Minnerup J, Meyer L, Madžar D, Reindl C, Madlener M, Malter M, Neumann B, Dimitriadis K; IGNITE Study Group
Epilepsia 2023 Jun;64(6):1482-1492. Epub 2023 Apr 16 doi: 10.1111/epi.17608. PMID: 37021609
When is it time for palliative and end-of-life care in status epilepticus?
Kälviäinen R, Allal Z, Kantanen AM
Epilepsy Behav 2023 Apr;141:109058. Epub 2023 Jan 4 doi: 10.1016/j.yebeh.2022.109058. PMID: 36604196
Predicting the progression to super-refractory status epilepticus: A machine-learning study.
Brigo F, Turcato G, Lattanzi S, Orlandi N, Turchi G, Zaboli A, Giovannini G, Meletti S
J Neurol Sci 2022 Dec 15;443:120481. Epub 2022 Oct 28 doi: 10.1016/j.jns.2022.120481. PMID: 36332322
Management of Status Epilepticus, Refractory Status Epilepticus, and Super-refractory Status Epilepticus.
Trinka E, Leitinger M
Continuum (Minneap Minn) 2022 Apr 1;28(2):559-602. doi: 10.1212/CON.0000000000001103. PMID: 35393970
Status Epilepticus, Refractory Status Epilepticus, and Super-refractory Status Epilepticus.
Nelson SE, Varelas PN
Continuum (Minneap Minn) 2018 Dec;24(6):1683-1707. doi: 10.1212/CON.0000000000000668. PMID: 30516601

Recent systematic reviews

Responsive neurostimulation as a treatment for super-refractory focal status epilepticus: a systematic review and case series.
Ernst LD, Raslan AM, Wabulya A, Shin HW, Cash SS, Yang JC, Sagi V, King-Stephens D, Damisah EC, Ramos A, Hussain B, Toprani S, Brandman DM, Shahlaie K, Kanth K, Arain A, Peters A, Rolston JD, Berns M, Patel SI, Uysal U
J Neurosurg 2024 Jan 1;140(1):201-209. Epub 2023 Jun 9 doi: 10.3171/2023.4.JNS23367. PMID: 37329518
Combined Responsive Neurostimulation and Focal Resection for Super Refractory Status Epilepticus: A Systematic Review and Illustrative Case Report.
Mamaril-Davis J, Vessell M, Ball T, Palade A, Shafer C, Aguilar-Salinas P, Fowler B, Mirro E, Neimat J, Sagi V, Bina RW
World Neurosurg 2022 Nov;167:195-204.e7. Epub 2022 Aug 7 doi: 10.1016/j.wneu.2022.07.141. PMID: 35948220
Considering safety and patient tolerance in the use of ketogenic diet in the management of refractory and super-refractory status epilepticus: a systematic review.
Dozières-Puyravel B, Höhn S, Auvin S
Expert Rev Neurother 2021 Nov;21(11):1303-1308. Epub 2021 Jul 24 doi: 10.1080/14737175.2021.1956905. PMID: 34275391
Vagus nerve stimulation in refractory and super-refractory status epilepticus - A systematic review.
Dibué-Adjei M, Brigo F, Yamamoto T, Vonck K, Trinka E
Brain Stimul 2019 Sep-Oct;12(5):1101-1110. Epub 2019 May 14 doi: 10.1016/j.brs.2019.05.011. PMID: 31126871
Topiramate in the Treatment of Generalized Convulsive Status Epilepticus in Adults: A Systematic Review with Individual Patient Data Analysis.
Brigo F, Bragazzi NL, Igwe SC, Nardone R, Trinka E
Drugs 2017 Jan;77(1):67-74. doi: 10.1007/s40265-016-0672-2. PMID: 28004305Free PMC Article

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