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Myoclonic-atonic epilepsy(MAE)

MedGen UID:
834110
Concept ID:
CN231318
Disease or Syndrome
Synonym: MAE
 
Gene (location): SLC6A1 (3p25.3)
 
Monarch Initiative: MONDO:0014633
OMIM®: 616421

Definition

Myoclonic-atonic epilepsy (MAE) is an autosomal dominant disorder characterized by onset of absence and myoclonic seizures in early childhood. Patients have delayed development before the onset of seizures and show varying degrees of impaired intellectual development following seizure onset (summary by Carvill et al., 2015). [from OMIM]

Term Hierarchy

Professional guidelines

PubMed

Menon RN, Nambiar PN, Keni RR, Baishya J, Radhakrishnan A, Cherian A, Nampoothiri S, Madhavilatha GK, Kotecha UH, Thomas SV
Neurol India 2021 Nov-Dec;69(6):1701-1705. doi: 10.4103/0028-3886.333508. PMID: 34979672

Recent clinical studies

Etiology

Whitney R, Komar M, Yoganathan S, Costain G, Jain P
Pediatr Neurol 2024 Feb;151:138-142. Epub 2023 Dec 14 doi: 10.1016/j.pediatrneurol.2023.12.006. PMID: 38157719
Kim SH, Seo J, Kwon SS, Teng LY, Won D, Shin S, Lee JS, Lee ST, Choi JR, Kang HC
Epilepsia 2024 Mar;65(3):766-778. Epub 2023 Dec 22 doi: 10.1111/epi.17857. PMID: 38073125
Mattison KA, Butler KM, Inglis GAS, Dayan O, Boussidan H, Bhambhani V, Philbrook B, da Silva C, Alexander JJ, Kanner BI, Escayg A
Epilepsia 2018 Sep;59(9):e135-e141. Epub 2018 Aug 21 doi: 10.1111/epi.14531. PMID: 30132828
Johannesen KM, Gardella E, Linnankivi T, Courage C, de Saint Martin A, Lehesjoki AE, Mignot C, Afenjar A, Lesca G, Abi-Warde MT, Chelly J, Piton A, Merritt JL 2nd, Rodan LH, Tan WH, Bird LM, Nespeca M, Gleeson JG, Yoo Y, Choi M, Chae JH, Czapansky-Beilman D, Reichert SC, Pendziwiat M, Verhoeven JS, Schelhaas HJ, Devinsky O, Christensen J, Specchio N, Trivisano M, Weber YG, Nava C, Keren B, Doummar D, Schaefer E, Hopkins S, Dubbs H, Shaw JE, Pisani L, Myers CT, Tang S, Tang S, Pal DK, Millichap JJ, Carvill GL, Helbig KL, Mecarelli O, Striano P, Helbig I, Rubboli G, Mefford HC, Møller RS
Epilepsia 2018 Feb;59(2):389-402. Epub 2018 Jan 8 doi: 10.1111/epi.13986. PMID: 29315614Free PMC Article
Wolff M, Johannesen KM, Hedrich UBS, Masnada S, Rubboli G, Gardella E, Lesca G, Ville D, Milh M, Villard L, Afenjar A, Chantot-Bastaraud S, Mignot C, Lardennois C, Nava C, Schwarz N, Gérard M, Perrin L, Doummar D, Auvin S, Miranda MJ, Hempel M, Brilstra E, Knoers N, Verbeek N, van Kempen M, Braun KP, Mancini G, Biskup S, Hörtnagel K, Döcker M, Bast T, Loddenkemper T, Wong-Kisiel L, Baumeister FM, Fazeli W, Striano P, Dilena R, Fontana E, Zara F, Kurlemann G, Klepper J, Thoene JG, Arndt DH, Deconinck N, Schmitt-Mechelke T, Maier O, Muhle H, Wical B, Finetti C, Brückner R, Pietz J, Golla G, Jillella D, Linnet KM, Charles P, Moog U, Õiglane-Shlik E, Mantovani JF, Park K, Deprez M, Lederer D, Mary S, Scalais E, Selim L, Van Coster R, Lagae L, Nikanorova M, Hjalgrim H, Korenke GC, Trivisano M, Specchio N, Ceulemans B, Dorn T, Helbig KL, Hardies K, Stamberger H, de Jonghe P, Weckhuysen S, Lemke JR, Krägeloh-Mann I, Helbig I, Kluger G, Lerche H, Møller RS
Brain 2017 May 1;140(5):1316-1336. doi: 10.1093/brain/awx054. PMID: 28379373

Diagnosis

Whitney R, Komar M, Yoganathan S, Costain G, Jain P
Pediatr Neurol 2024 Feb;151:138-142. Epub 2023 Dec 14 doi: 10.1016/j.pediatrneurol.2023.12.006. PMID: 38157719
Kim SH, Seo J, Kwon SS, Teng LY, Won D, Shin S, Lee JS, Lee ST, Choi JR, Kang HC
Epilepsia 2024 Mar;65(3):766-778. Epub 2023 Dec 22 doi: 10.1111/epi.17857. PMID: 38073125
Routier L, Verny F, Barcia G, Chemaly N, Desguerre I, Colleaux L, Nabbout R
Clin Genet 2019 Sep;96(3):254-260. Epub 2019 Jun 6 doi: 10.1111/cge.13581. PMID: 31170314
Mattison KA, Butler KM, Inglis GAS, Dayan O, Boussidan H, Bhambhani V, Philbrook B, da Silva C, Alexander JJ, Kanner BI, Escayg A
Epilepsia 2018 Sep;59(9):e135-e141. Epub 2018 Aug 21 doi: 10.1111/epi.14531. PMID: 30132828
Johannesen KM, Gardella E, Linnankivi T, Courage C, de Saint Martin A, Lehesjoki AE, Mignot C, Afenjar A, Lesca G, Abi-Warde MT, Chelly J, Piton A, Merritt JL 2nd, Rodan LH, Tan WH, Bird LM, Nespeca M, Gleeson JG, Yoo Y, Choi M, Chae JH, Czapansky-Beilman D, Reichert SC, Pendziwiat M, Verhoeven JS, Schelhaas HJ, Devinsky O, Christensen J, Specchio N, Trivisano M, Weber YG, Nava C, Keren B, Doummar D, Schaefer E, Hopkins S, Dubbs H, Shaw JE, Pisani L, Myers CT, Tang S, Tang S, Pal DK, Millichap JJ, Carvill GL, Helbig KL, Mecarelli O, Striano P, Helbig I, Rubboli G, Mefford HC, Møller RS
Epilepsia 2018 Feb;59(2):389-402. Epub 2018 Jan 8 doi: 10.1111/epi.13986. PMID: 29315614Free PMC Article

Therapy

Caraballo RH, Reyes Valenzuela G, Fortini S, Espeche A, Gamboni B, Bautista C, Cachia P, Semprino M, Gallo A, Galicchio S
Epilepsy Behav 2022 Jun;131(Pt A):108702. Epub 2022 May 5 doi: 10.1016/j.yebeh.2022.108702. PMID: 35526461
Johannesen KM, Gardella E, Linnankivi T, Courage C, de Saint Martin A, Lehesjoki AE, Mignot C, Afenjar A, Lesca G, Abi-Warde MT, Chelly J, Piton A, Merritt JL 2nd, Rodan LH, Tan WH, Bird LM, Nespeca M, Gleeson JG, Yoo Y, Choi M, Chae JH, Czapansky-Beilman D, Reichert SC, Pendziwiat M, Verhoeven JS, Schelhaas HJ, Devinsky O, Christensen J, Specchio N, Trivisano M, Weber YG, Nava C, Keren B, Doummar D, Schaefer E, Hopkins S, Dubbs H, Shaw JE, Pisani L, Myers CT, Tang S, Tang S, Pal DK, Millichap JJ, Carvill GL, Helbig KL, Mecarelli O, Striano P, Helbig I, Rubboli G, Mefford HC, Møller RS
Epilepsia 2018 Feb;59(2):389-402. Epub 2018 Jan 8 doi: 10.1111/epi.13986. PMID: 29315614Free PMC Article
Wolff M, Johannesen KM, Hedrich UBS, Masnada S, Rubboli G, Gardella E, Lesca G, Ville D, Milh M, Villard L, Afenjar A, Chantot-Bastaraud S, Mignot C, Lardennois C, Nava C, Schwarz N, Gérard M, Perrin L, Doummar D, Auvin S, Miranda MJ, Hempel M, Brilstra E, Knoers N, Verbeek N, van Kempen M, Braun KP, Mancini G, Biskup S, Hörtnagel K, Döcker M, Bast T, Loddenkemper T, Wong-Kisiel L, Baumeister FM, Fazeli W, Striano P, Dilena R, Fontana E, Zara F, Kurlemann G, Klepper J, Thoene JG, Arndt DH, Deconinck N, Schmitt-Mechelke T, Maier O, Muhle H, Wical B, Finetti C, Brückner R, Pietz J, Golla G, Jillella D, Linnet KM, Charles P, Moog U, Õiglane-Shlik E, Mantovani JF, Park K, Deprez M, Lederer D, Mary S, Scalais E, Selim L, Van Coster R, Lagae L, Nikanorova M, Hjalgrim H, Korenke GC, Trivisano M, Specchio N, Ceulemans B, Dorn T, Helbig KL, Hardies K, Stamberger H, de Jonghe P, Weckhuysen S, Lemke JR, Krägeloh-Mann I, Helbig I, Kluger G, Lerche H, Møller RS
Brain 2017 May 1;140(5):1316-1336. doi: 10.1093/brain/awx054. PMID: 28379373

Prognosis

Tzartos J, Pechlivanidou M, Bosveli D, Ninou E, Yuceer H, Yalcin B, Kucukali CI, Tuzun E, Tzartos S, Turkdogan D
In Vivo 2024 Jan-Feb;38(1):351-357. doi: 10.21873/invivo.13445. PMID: 38148071Free PMC Article
Mermer F, Poliquin S, Zhou S, Wang X, Ding Y, Yin F, Shen W, Wang J, Rigsby K, Xu D, Mack T, Nwosu G, Flamm C, Stein M, Kang JQ
Neurobiol Dis 2022 Oct 1;172:105810. Epub 2022 Jul 14 doi: 10.1016/j.nbd.2022.105810. PMID: 35840120Free PMC Article
Menon RN, Nambiar PN, Keni RR, Baishya J, Radhakrishnan A, Cherian A, Nampoothiri S, Madhavilatha GK, Kotecha UH, Thomas SV
Neurol India 2021 Nov-Dec;69(6):1701-1705. doi: 10.4103/0028-3886.333508. PMID: 34979672
Breuillard D, Jambaqué I, Laschet J, Nabbout R
Epilepsy Behav 2019 Oct;99:106482. Epub 2019 Aug 25 doi: 10.1016/j.yebeh.2019.106482. PMID: 31461681
Wolff M, Johannesen KM, Hedrich UBS, Masnada S, Rubboli G, Gardella E, Lesca G, Ville D, Milh M, Villard L, Afenjar A, Chantot-Bastaraud S, Mignot C, Lardennois C, Nava C, Schwarz N, Gérard M, Perrin L, Doummar D, Auvin S, Miranda MJ, Hempel M, Brilstra E, Knoers N, Verbeek N, van Kempen M, Braun KP, Mancini G, Biskup S, Hörtnagel K, Döcker M, Bast T, Loddenkemper T, Wong-Kisiel L, Baumeister FM, Fazeli W, Striano P, Dilena R, Fontana E, Zara F, Kurlemann G, Klepper J, Thoene JG, Arndt DH, Deconinck N, Schmitt-Mechelke T, Maier O, Muhle H, Wical B, Finetti C, Brückner R, Pietz J, Golla G, Jillella D, Linnet KM, Charles P, Moog U, Õiglane-Shlik E, Mantovani JF, Park K, Deprez M, Lederer D, Mary S, Scalais E, Selim L, Van Coster R, Lagae L, Nikanorova M, Hjalgrim H, Korenke GC, Trivisano M, Specchio N, Ceulemans B, Dorn T, Helbig KL, Hardies K, Stamberger H, de Jonghe P, Weckhuysen S, Lemke JR, Krägeloh-Mann I, Helbig I, Kluger G, Lerche H, Møller RS
Brain 2017 May 1;140(5):1316-1336. doi: 10.1093/brain/awx054. PMID: 28379373

Clinical prediction guides

Mermer F, Poliquin S, Zhou S, Wang X, Ding Y, Yin F, Shen W, Wang J, Rigsby K, Xu D, Mack T, Nwosu G, Flamm C, Stein M, Kang JQ
Neurobiol Dis 2022 Oct 1;172:105810. Epub 2022 Jul 14 doi: 10.1016/j.nbd.2022.105810. PMID: 35840120Free PMC Article
Caraballo RH, Reyes Valenzuela G, Fortini S, Espeche A, Gamboni B, Bautista C, Cachia P, Semprino M, Gallo A, Galicchio S
Epilepsy Behav 2022 Jun;131(Pt A):108702. Epub 2022 May 5 doi: 10.1016/j.yebeh.2022.108702. PMID: 35526461
Semino F, Schröter J, Willemsen MH, Bast T, Biskup S, Beck-Woedl S, Brennenstuhl H, Schaaf CP, Kölker S, Hoffmann GF, Haack TB, Syrbe S
Hum Mutat 2021 Sep;42(9):1094-1100. Epub 2021 Jul 5 doi: 10.1002/humu.24245. PMID: 34157790
Breuillard D, Jambaqué I, Laschet J, Nabbout R
Epilepsy Behav 2019 Oct;99:106482. Epub 2019 Aug 25 doi: 10.1016/j.yebeh.2019.106482. PMID: 31461681
Johannesen KM, Gardella E, Linnankivi T, Courage C, de Saint Martin A, Lehesjoki AE, Mignot C, Afenjar A, Lesca G, Abi-Warde MT, Chelly J, Piton A, Merritt JL 2nd, Rodan LH, Tan WH, Bird LM, Nespeca M, Gleeson JG, Yoo Y, Choi M, Chae JH, Czapansky-Beilman D, Reichert SC, Pendziwiat M, Verhoeven JS, Schelhaas HJ, Devinsky O, Christensen J, Specchio N, Trivisano M, Weber YG, Nava C, Keren B, Doummar D, Schaefer E, Hopkins S, Dubbs H, Shaw JE, Pisani L, Myers CT, Tang S, Tang S, Pal DK, Millichap JJ, Carvill GL, Helbig KL, Mecarelli O, Striano P, Helbig I, Rubboli G, Mefford HC, Møller RS
Epilepsia 2018 Feb;59(2):389-402. Epub 2018 Jan 8 doi: 10.1111/epi.13986. PMID: 29315614Free PMC Article

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