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Focal impaired awareness myoclonic seizure

MedGen UID:
1710318
•Concept ID:
C5397740
•
Disease or Syndrome
Synonyms: Focal onset impaired awareness myoclonic epileptic seizure; Focal onset impaired awareness myoclonic seizure; Focal-onset impaired awareness myoclonic epileptic seizure
SNOMED CT: Focal-onset impaired awareness myoclonic epileptic seizure (1269370000); Focal onset impaired awareness myoclonic epileptic seizure (1269370000); Focal onset impaired awareness myoclonic seizure (1269370000); Focal impaired awareness myoclonic seizure (1269370000)
 
HPO: HP:0032730

Definition

A focal myoclonic seizure in which awareness is partially or fully impaired at some point during the seizure. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFocal impaired awareness myoclonic seizure

Professional guidelines

PubMed

Diagnosis, treatment, and follow-up of patients with cerebral amyloid angiopathy-related inflammation.
Cancelloni V, Rufa A, Battisti C, De Stefano N, Mastrocinque E, Garosi G, Venezia D, Chiarotti I, Cerase A
Neurol Sci 2022 Nov;43(11):6381-6387. Epub 2022 Aug 5 doi: 10.1007/s10072-022-06299-y. PMID: 35930182Free PMC Article
Testing awareness in focal seizures: Clinical practice and interpretation of current guidelines.
Contreras Ramirez V, Vaddiparti A, Blumenfeld H
Ann Clin Transl Neurol 2022 May;9(5):762-765. Epub 2022 Apr 29 doi: 10.1002/acn3.51552. PMID: 35485201Free PMC Article
Encephalocele-Associated Drug-Resistant Epilepsy of Adult Onset: Diagnosis, Management, and Outcomes.
Ramos-Fresnedo A, Domingo RA, McGeary RC, Sirven JI, Feyissa AM, Tatum W, Ritaccio AL, Middlebrooks EH, Grewal SS
World Neurosurg 2021 Jul;151:91-101. Epub 2021 May 5 doi: 10.1016/j.wneu.2021.04.121. PMID: 33964498

Recent clinical studies

Etiology

Focal epilepsies: Update on diagnosis and classification.
Nascimento FA, Friedman D, Peters JM, Bensalem-Owen MK, Cendes F, Rampp S, Wirrell E, Blümcke I, Tatum W, Beniczky S
Epileptic Disord 2023 Feb;25(1):1-17. Epub 2023 Apr 17 doi: 10.1002/epd2.20045. PMID: 36938903
Randomized phase 2 study of adjunctive cenobamate in patients with uncontrolled focal seizures.
Chung SS, French JA, Kowalski J, Krauss GL, Lee SK, Maciejowski M, Rosenfeld WE, Sperling MR, Mizne S, Kamin M
Neurology 2020 Jun 2;94(22):e2311-e2322. Epub 2020 May 14 doi: 10.1212/WNL.0000000000009530. PMID: 32409485Free PMC Article
Safety and efficacy of adjunctive cenobamate (YKP3089) in patients with uncontrolled focal seizures: a multicentre, double-blind, randomised, placebo-controlled, dose-response trial.
Krauss GL, Klein P, Brandt C, Lee SK, Milanov I, Milovanovic M, Steinhoff BJ, Kamin M
Lancet Neurol 2020 Jan;19(1):38-48. Epub 2019 Nov 14 doi: 10.1016/S1474-4422(19)30399-0. PMID: 31734103
Social cognition and psychopathology in childhood and adolescence.
Besag FMC, Vasey MJ
Epilepsy Behav 2019 Nov;100(Pt B):106210. Epub 2019 Jun 10 doi: 10.1016/j.yebeh.2019.03.015. PMID: 31196824
Consciousness of seizures and consciousness during seizures: are they related?
Detyniecki K, Blumenfeld H
Epilepsy Behav 2014 Jan;30:6-9. Epub 2013 Oct 12 doi: 10.1016/j.yebeh.2013.09.018. PMID: 24126026Free PMC Article

Diagnosis

Diabetes ketoacidosis and Recurrent Childhood Stroke-like Episodes.
Liu RC, Sheu JN, Liu CS, Tsai JD
Acta Neurol Taiwan 2024 Mar 30;33(1):41-42. PMID: 37853548
Clinical and epilepsy characteristics in Wolf-Hirschhorn syndrome (4p-): A review.
Paprocka J, Kaminiów K, Yetkin O, Tekturk P, Baykan B, Leiz S, Kluger G, Striano P
Seizure 2024 Mar;116:14-23. Epub 2022 Dec 8 doi: 10.1016/j.seizure.2022.12.001. PMID: 36526544
Focal epilepsies: Update on diagnosis and classification.
Nascimento FA, Friedman D, Peters JM, Bensalem-Owen MK, Cendes F, Rampp S, Wirrell E, Blümcke I, Tatum W, Beniczky S
Epileptic Disord 2023 Feb;25(1):1-17. Epub 2023 Apr 17 doi: 10.1002/epd2.20045. PMID: 36938903
CASPR2-IgG-associated autoimmune seizures.
Garrido Sanabria ER, Zahid A, Britton J, Kraus GJ, López-Chiriboga AS, Zekeridou A, Flanagan EP, McKeon A, Mills JR, Pittock SJ, Dubey D
Epilepsia 2022 Mar;63(3):709-722. Epub 2022 Jan 15 doi: 10.1111/epi.17164. PMID: 35032032
Randomized phase 2 study of adjunctive cenobamate in patients with uncontrolled focal seizures.
Chung SS, French JA, Kowalski J, Krauss GL, Lee SK, Maciejowski M, Rosenfeld WE, Sperling MR, Mizne S, Kamin M
Neurology 2020 Jun 2;94(22):e2311-e2322. Epub 2020 May 14 doi: 10.1212/WNL.0000000000009530. PMID: 32409485Free PMC Article

Therapy

CASPR2-IgG-associated autoimmune seizures.
Garrido Sanabria ER, Zahid A, Britton J, Kraus GJ, López-Chiriboga AS, Zekeridou A, Flanagan EP, McKeon A, Mills JR, Pittock SJ, Dubey D
Epilepsia 2022 Mar;63(3):709-722. Epub 2022 Jan 15 doi: 10.1111/epi.17164. PMID: 35032032
Adult phenotype of KCNQ2 encephalopathy.
Boets S, Johannesen KM, Destree A, Manti F, Ramantani G, Lesca G, Vercueil L, Koenig MK, Striano P, Møller RS, Cooper E, Weckhuysen S
J Med Genet 2022 Jun;59(6):528-535. Epub 2021 Apr 2 doi: 10.1136/jmedgenet-2020-107449. PMID: 33811133
Randomized phase 2 study of adjunctive cenobamate in patients with uncontrolled focal seizures.
Chung SS, French JA, Kowalski J, Krauss GL, Lee SK, Maciejowski M, Rosenfeld WE, Sperling MR, Mizne S, Kamin M
Neurology 2020 Jun 2;94(22):e2311-e2322. Epub 2020 May 14 doi: 10.1212/WNL.0000000000009530. PMID: 32409485Free PMC Article
Safety and efficacy of adjunctive cenobamate (YKP3089) in patients with uncontrolled focal seizures: a multicentre, double-blind, randomised, placebo-controlled, dose-response trial.
Krauss GL, Klein P, Brandt C, Lee SK, Milanov I, Milovanovic M, Steinhoff BJ, Kamin M
Lancet Neurol 2020 Jan;19(1):38-48. Epub 2019 Nov 14 doi: 10.1016/S1474-4422(19)30399-0. PMID: 31734103
Seizure onset predicts its type.
Donos C, Maliia MD, Dümpelmann M, Schulze-Bonhage A
Epilepsia 2018 Mar;59(3):650-660. Epub 2018 Jan 11 doi: 10.1111/epi.13997. PMID: 29322500

Prognosis

Distinct signatures of loss of consciousness in focal impaired awareness versus tonic-clonic seizures.
Juan E, Górska U, Kozma C, Papantonatos C, Bugnon T, Denis C, Kremen V, Worrell G, Struck AF, Bateman LM, Merricks EM, Blumenfeld H, Tononi G, Schevon C, Boly M
Brain 2023 Jan 5;146(1):109-123. doi: 10.1093/brain/awac291. PMID: 36383415Free PMC Article
PIGN encephalopathy: Characterizing the epileptology.
Bayat A, de Valles-Ibáñez G, Pendziwiat M, Knaus A, Alt K, Biamino E, Bley A, Calvert S, Carney P, Caro-Llopis A, Ceulemans B, Cousin J, Davis S, des Portes V, Edery P, England E, Ferreira C, Freeman J, Gener B, Gorce M, Heron D, Hildebrand MS, Jezela-Stanek A, Jouk PS, Keren B, Kloth K, Kluger G, Kuhn M, Lemke JR, Li H, Martinez F, Maxton C, Mefford HC, Merla G, Mierzewska H, Muir A, Monfort S, Nicolai J, Norman J, O'Grady G, Oleksy B, Orellana C, Orec LE, Peinhardt C, Pronicka E, Rosello M, Santos-Simarro F, Schwaibold EMC, Stegmann APA, Stumpel CT, Szczepanik E, Terczyńska I, Thevenon J, Tzschach A, Van Bogaert P, Vittorini R, Walsh S, Weckhuysen S, Weissman B, Wolfe L, Reymond A, De Nittis P, Poduri A, Olson H, Striano P, Lesca G, Scheffer IE, Møller RS, Sadleir LG
Epilepsia 2022 Apr;63(4):974-991. Epub 2022 Feb 18 doi: 10.1111/epi.17173. PMID: 35179230
CASPR2-IgG-associated autoimmune seizures.
Garrido Sanabria ER, Zahid A, Britton J, Kraus GJ, López-Chiriboga AS, Zekeridou A, Flanagan EP, McKeon A, Mills JR, Pittock SJ, Dubey D
Epilepsia 2022 Mar;63(3):709-722. Epub 2022 Jan 15 doi: 10.1111/epi.17164. PMID: 35032032
Adult phenotype of KCNQ2 encephalopathy.
Boets S, Johannesen KM, Destree A, Manti F, Ramantani G, Lesca G, Vercueil L, Koenig MK, Striano P, Møller RS, Cooper E, Weckhuysen S
J Med Genet 2022 Jun;59(6):528-535. Epub 2021 Apr 2 doi: 10.1136/jmedgenet-2020-107449. PMID: 33811133
Seizure onset predicts its type.
Donos C, Maliia MD, Dümpelmann M, Schulze-Bonhage A
Epilepsia 2018 Mar;59(3):650-660. Epub 2018 Jan 11 doi: 10.1111/epi.13997. PMID: 29322500

Clinical prediction guides

Diabetes ketoacidosis and Recurrent Childhood Stroke-like Episodes.
Liu RC, Sheu JN, Liu CS, Tsai JD
Acta Neurol Taiwan 2024 Mar 30;33(1):41-42. PMID: 37853548
PIGN encephalopathy: Characterizing the epileptology.
Bayat A, de Valles-Ibáñez G, Pendziwiat M, Knaus A, Alt K, Biamino E, Bley A, Calvert S, Carney P, Caro-Llopis A, Ceulemans B, Cousin J, Davis S, des Portes V, Edery P, England E, Ferreira C, Freeman J, Gener B, Gorce M, Heron D, Hildebrand MS, Jezela-Stanek A, Jouk PS, Keren B, Kloth K, Kluger G, Kuhn M, Lemke JR, Li H, Martinez F, Maxton C, Mefford HC, Merla G, Mierzewska H, Muir A, Monfort S, Nicolai J, Norman J, O'Grady G, Oleksy B, Orellana C, Orec LE, Peinhardt C, Pronicka E, Rosello M, Santos-Simarro F, Schwaibold EMC, Stegmann APA, Stumpel CT, Szczepanik E, Terczyńska I, Thevenon J, Tzschach A, Van Bogaert P, Vittorini R, Walsh S, Weckhuysen S, Weissman B, Wolfe L, Reymond A, De Nittis P, Poduri A, Olson H, Striano P, Lesca G, Scheffer IE, Møller RS, Sadleir LG
Epilepsia 2022 Apr;63(4):974-991. Epub 2022 Feb 18 doi: 10.1111/epi.17173. PMID: 35179230
Focal impaired awareness seizures in a rodent model: A functional anatomy.
Adotevi N, Kapur J
Epilepsia Open 2022 Mar;7(1):110-123. Epub 2021 Dec 17 doi: 10.1002/epi4.12563. PMID: 34822222Free PMC Article
The Revised Definition and Classification of Epilepsy for Neurodiagnostic Technologists.
Fisher RS, Bonner AM
Neurodiagn J 2018;58(1):1-10. doi: 10.1080/21646821.2018.1428455. PMID: 29562876
Seizure onset predicts its type.
Donos C, Maliia MD, Dümpelmann M, Schulze-Bonhage A
Epilepsia 2018 Mar;59(3):650-660. Epub 2018 Jan 11 doi: 10.1111/epi.13997. PMID: 29322500

Recent systematic reviews

Clinical and epilepsy characteristics in Wolf-Hirschhorn syndrome (4p-): A review.
Paprocka J, Kaminiów K, Yetkin O, Tekturk P, Baykan B, Leiz S, Kluger G, Striano P
Seizure 2024 Mar;116:14-23. Epub 2022 Dec 8 doi: 10.1016/j.seizure.2022.12.001. PMID: 36526544
Dyke-Davidoff-Masson Syndrome: Main clinical and radiological findings- systematic literature review.
Rondão MBA, Hsu BRRHS, Centeno RS, de Aguiar PHP
Seizure 2023 Aug;110:58-68. Epub 2023 Jun 3 doi: 10.1016/j.seizure.2023.04.020. PMID: 37327751
Posterior Cingulate Epilepsy: A Systematic Review.
Pepin C, Brochu K, Lessard-Bonaventure P, Nguyen DK, Martineau L
J Clin Neurophysiol 2023 Sep 1;40(6):507-515. Epub 2023 Mar 15 doi: 10.1097/WNP.0000000000000975. PMID: 36930208
A systematic review of adults with Dravet syndrome.
Selvarajah A, Zulfiqar-Ali Q, Marques P, Rong M, Andrade DM
Seizure 2021 Apr;87:39-45. Epub 2021 Feb 22 doi: 10.1016/j.seizure.2021.02.025. PMID: 33677403
Anti-seizure medications and efficacy against focal to bilateral tonic-clonic seizures: A systematic review with relevance for SUDEP prevention.
Cutillo G, Tolba H, Hirsch LJ
Epilepsy Behav 2021 Apr;117:107815. Epub 2021 Feb 26 doi: 10.1016/j.yebeh.2021.107815. PMID: 33640562

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