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Focal impaired awareness epileptic spasm

MedGen UID:
1711328
•Concept ID:
C5397850
•
Disease or Syndrome
HPO: HP:0032844

Definition

A type of focal-onset epileptic spasm in which awareness is impaired at some point during the seizure. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFocal impaired awareness epileptic spasm

Recent clinical studies

Etiology

ATP1A2-related epileptic encephalopathy and movement disorder: Clinical features of three novel patients.
Córdoba NM, Lince-Rivera I, Gómez JLR, Rubboli G, De la Rosa SO
Epileptic Disord 2024 Jun;26(3):332-340. Epub 2024 Mar 21 doi: 10.1002/epd2.20220. PMID: 38512072
Epilepsy in neurofibromatosis type 1: Diffuse cerebral dysfunction?
Serdaroglu E, Konuskan B, Karli Oguz K, Gurler G, Yalnizoglu D, Anlar B
Epilepsy Behav 2019 Sep;98(Pt A):6-9. Epub 2019 Jul 9 doi: 10.1016/j.yebeh.2019.06.022. PMID: 31299535
Risk factors of cognitive impairment in pediatric epilepsy patients with focal cortical dysplasia.
Kimura N, Takahashi Y, Shigematsu H, Imai K, Ikeda H, Ootani H, Takayama R, Mogami Y, Kimura N, Baba K, Matsuda K, Tottori T, Usui N, Kondou S, Inoue Y
Brain Dev 2019 Jan;41(1):77-84. Epub 2018 Jul 31 doi: 10.1016/j.braindev.2018.07.014. PMID: 30075883

Diagnosis

Duration of epileptic seizure types: A data-driven approach.
Meritam Larsen P, Wüstenhagen S, Terney D, Gardella E, Aurlien H, Beniczky S
Epilepsia 2023 Feb;64(2):469-478. Epub 2023 Jan 3 doi: 10.1111/epi.17492. PMID: 36597206Free PMC Article
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
Epilepsy in neurofibromatosis type 1: Diffuse cerebral dysfunction?
Serdaroglu E, Konuskan B, Karli Oguz K, Gurler G, Yalnizoglu D, Anlar B
Epilepsy Behav 2019 Sep;98(Pt A):6-9. Epub 2019 Jul 9 doi: 10.1016/j.yebeh.2019.06.022. PMID: 31299535

Therapy

ATP1A2-related epileptic encephalopathy and movement disorder: Clinical features of three novel patients.
Córdoba NM, Lince-Rivera I, Gómez JLR, Rubboli G, De la Rosa SO
Epileptic Disord 2024 Jun;26(3):332-340. Epub 2024 Mar 21 doi: 10.1002/epd2.20220. PMID: 38512072
Long-term safety and effectiveness of stiripentol in patients with Dravet syndrome: Interim report of a post-marketing surveillance study in Japan.
Yamada M, Suzuki K, Matsui D, Inoue Y, Ohtsuka Y
Epilepsy Res 2021 Feb;170:106535. Epub 2020 Dec 31 doi: 10.1016/j.eplepsyres.2020.106535. PMID: 33388609

Prognosis

Risk factors of cognitive impairment in pediatric epilepsy patients with focal cortical dysplasia.
Kimura N, Takahashi Y, Shigematsu H, Imai K, Ikeda H, Ootani H, Takayama R, Mogami Y, Kimura N, Baba K, Matsuda K, Tottori T, Usui N, Kondou S, Inoue Y
Brain Dev 2019 Jan;41(1):77-84. Epub 2018 Jul 31 doi: 10.1016/j.braindev.2018.07.014. PMID: 30075883

Clinical prediction guides

Duration of epileptic seizure types: A data-driven approach.
Meritam Larsen P, Wüstenhagen S, Terney D, Gardella E, Aurlien H, Beniczky S
Epilepsia 2023 Feb;64(2):469-478. Epub 2023 Jan 3 doi: 10.1111/epi.17492. PMID: 36597206Free PMC Article
Long-term safety and effectiveness of stiripentol in patients with Dravet syndrome: Interim report of a post-marketing surveillance study in Japan.
Yamada M, Suzuki K, Matsui D, Inoue Y, Ohtsuka Y
Epilepsy Res 2021 Feb;170:106535. Epub 2020 Dec 31 doi: 10.1016/j.eplepsyres.2020.106535. PMID: 33388609

Recent systematic reviews

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

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