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Developmental and epileptic encephalopathy 115

MedGen UID:
1052936
Concept ID:
CN377534
Disease or Syndrome
Synonym: developmental and epileptic encephalopathy 115
 
Gene (location): SNF8 (17q21.32)
 
Monarch Initiative: MONDO:0968946
OMIM®: 620783

Definition

Developmental and epileptic encephalopathy-115 (DEE115) is an autosomal recessive disorder characterized by severe developmental delay and epileptic encephalopathy, massive reduction of white matter, hypo-/aplasia of the corpus callosum, neurodevelopmental arrest, and early death (Brugger et al., 2024). For general phenotypic information and a discussion of genetic heterogeneity of DEE, see 308350. [from OMIM]

Recent clinical studies

Etiology

Poke G, Stanley J, Scheffer IE, Sadleir LG
Neurology 2023 Mar 28;100(13):e1363-e1375. Epub 2022 Dec 29 doi: 10.1212/WNL.0000000000206758. PMID: 36581463Free PMC Article
Kopciuch D, Barciszewska AM, Fliciński J, Zaprutko T, Kus K, Steinborn B, Nowakowska E
Acta Neurol Belg 2020 Feb;120(1):115-122. Epub 2019 Nov 13 doi: 10.1007/s13760-019-01236-x. PMID: 31721076
Kunduri G, Turner-Evans D, Konya Y, Izumi Y, Nagashima K, Lockett S, Holthuis J, Bamba T, Acharya U, Acharya JK
Proc Natl Acad Sci U S A 2018 Sep 18;115(38):E8919-E8928. Epub 2018 Sep 5 doi: 10.1073/pnas.1808463115. PMID: 30185559Free PMC Article
Richards KL, Milligan CJ, Richardson RJ, Jancovski N, Grunnet M, Jacobson LH, Undheim EAB, Mobli M, Chow CY, Herzig V, Csoti A, Panyi G, Reid CA, King GF, Petrou S
Proc Natl Acad Sci U S A 2018 Aug 21;115(34):E8077-E8085. Epub 2018 Aug 3 doi: 10.1073/pnas.1804764115. PMID: 30076230Free PMC Article
Lochy A, Jacques C, Maillard L, Colnat-Coulbois S, Rossion B, Jonas J
Proc Natl Acad Sci U S A 2018 Aug 7;115(32):E7595-E7604. Epub 2018 Jul 23 doi: 10.1073/pnas.1718987115. PMID: 30038000Free PMC Article

Diagnosis

Chen Y, Dawes R, Kim HC, Ljungdahl A, Stenton SL, Walker S, Lord J, Lemire G, Martin-Geary AC, Ganesh VS, Ma J, Ellingford JM, Delage E, D'Souza EN, Dong S, Adams DR, Allan K, Bakshi M, Baldwin EE, Berger SI, Bernstein JA, Bhatnagar I, Blair E, Brown NJ, Burrage LC, Chapman K, Coman DJ, Compton AG, Cunningham CA, D'Souza P, Danecek P, Délot EC, Dias KR, Elias ER, Elmslie F, Evans CA, Ewans L, Ezell K, Fraser JL, Gallacher L, Genetti CA, Goriely A, Grant CL, Haack T, Higgs JE, Hinch AG, Hurles ME, Kuechler A, Lachlan KL, Lalani SR, Lecoquierre F, Leitão E, Fevre AL, Leventer RJ, Liebelt JE, Lindsay S, Lockhart PJ, Ma AS, Macnamara EF, Mansour S, Maurer TM, Mendez HR, Metcalfe K, Montgomery SB, Moosajee M, Nassogne MC, Neumann S, O'Donoghue M, O'Leary M, Palmer EE, Pattani N, Phillips J, Pitsava G, Pysar R, Rehm HL, Reuter CM, Revencu N, Riess A, Rius R, Rodan L, Roscioli T, Rosenfeld JA, Sachdev R, Shaw-Smith CJ, Simons C, Sisodiya SM, Snell P, St Clair L, Stark Z, Stewart HS, Tan TY, Tan NB, Temple SEL, Thorburn DR, Tifft CJ, Uebergang E, VanNoy GE, Vasudevan P, Vilain E, Viskochil DH, Wedd L, Wheeler MT, White SM, Wojcik M, Wolfe LA, Wolfenson Z, Wright CF, Xiao C, Zocche D, Rubenstein JL, Markenscoff-Papadimitriou E, Fica SM, Baralle D, Depienne C, MacArthur DG, Howson JMM, Sanders SJ, O'Donnell-Luria A, Whiffin N
Nature 2024 Aug;632(8026):832-840. Epub 2024 Jul 11 doi: 10.1038/s41586-024-07773-7. PMID: 38991538Free PMC Article
Poke G, Stanley J, Scheffer IE, Sadleir LG
Neurology 2023 Mar 28;100(13):e1363-e1375. Epub 2022 Dec 29 doi: 10.1212/WNL.0000000000206758. PMID: 36581463Free PMC Article
Saleh S, Beyyumi E, Al Kaabi A, Hertecant J, Barakat D, Al Dhaheri NS, Al-Gazali L, Al Shamsi A
Clin Genet 2021 Nov;100(5):573-600. Epub 2021 Aug 19 doi: 10.1111/cge.14044. PMID: 34374989
Takata A, Nakashima M, Saitsu H, Mizuguchi T, Mitsuhashi S, Takahashi Y, Okamoto N, Osaka H, Nakamura K, Tohyama J, Haginoya K, Takeshita S, Kuki I, Okanishi T, Goto T, Sasaki M, Sakai Y, Miyake N, Miyatake S, Tsuchida N, Iwama K, Minase G, Sekiguchi F, Fujita A, Imagawa E, Koshimizu E, Uchiyama Y, Hamanaka K, Ohba C, Itai T, Aoi H, Saida K, Sakaguchi T, Den K, Takahashi R, Ikeda H, Yamaguchi T, Tsukamoto K, Yoshitomi S, Oboshi T, Imai K, Kimizu T, Kobayashi Y, Kubota M, Kashii H, Baba S, Iai M, Kira R, Hara M, Ohta M, Miyata Y, Miyata R, Takanashi JI, Matsui J, Yokochi K, Shimono M, Amamoto M, Takayama R, Hirabayashi S, Aiba K, Matsumoto H, Nabatame S, Shiihara T, Kato M, Matsumoto N
Nat Commun 2019 Jun 7;10(1):2506. doi: 10.1038/s41467-019-10482-9. PMID: 31175295Free PMC Article
Lochy A, Jacques C, Maillard L, Colnat-Coulbois S, Rossion B, Jonas J
Proc Natl Acad Sci U S A 2018 Aug 7;115(32):E7595-E7604. Epub 2018 Jul 23 doi: 10.1073/pnas.1718987115. PMID: 30038000Free PMC Article

Therapy

Raga SV, Essajee F, Solomons R, Van Toorn R, Wilmshurst JM
Dev Med Child Neurol 2023 Apr;65(4):526-533. Epub 2022 Oct 13 doi: 10.1111/dmcn.15433. PMID: 36229895
Kuchenbuch M, Benquet P, Kaminska A, Roubertie A, Carme E, de Saint Martin A, Hirsch E, Dubois F, Laroche C, Barcia G, Chemaly N, Milh M, Villeneuve N, Sauleau P, Modolo J, Wendling F, Nabbout R
Epilepsia 2019 Jan;60(1):20-32. Epub 2018 Dec 7 doi: 10.1111/epi.14605. PMID: 30525185
Spagnoli C, Falsaperla R, Deolmi M, Corsello G, Pisani F
Ital J Pediatr 2018 Nov 1;44(1):115. doi: 10.1186/s13052-018-0573-y. PMID: 30382869Free PMC Article
Tacke M, Borggraefe I, Gerstl L, Heinen F, Vill K, Bonfert M, Bast T; HEAD Study group, Neubauer BA, Baumeister F, Baethmann M, Bentele K, Blank C, Blank HM, Bode H, Bosch F, Brandl U, Brockmann K, Dahlem P, Ernst JP, Feldmann E, Fiedler A, Gerigk M, Heß S, Hikel C, Hoffmann HG, Kieslich M, Klepper J, Kluger G, Koch H, Koch W, Korinthenberg R, Krois I, Kühne H, Kurlemann G, Mandl M, Mause U, Navratil P, Opp J, Penzien J, Prietsch V, Quattländer A, Rating D, Schara U, Shamdeen MG, Sprinz A, Wendker-Magrabi H, Stephani U, Muhle H, Straßburg HM, Töpke B, Trollmann R, Tuschen-Hofstätter E, Waltz S, Weber G, Wien FU, Wolff M, Polster T, Freitag H, Sönmez Ö, Reinhardt K, Traus M, Hoovey Z
Seizure 2018 Mar;56:115-120. Epub 2018 Feb 3 doi: 10.1016/j.seizure.2018.01.015. PMID: 29475094
Jonas R, Nguyen S, Hu B, Asarnow RF, LoPresti C, Curtiss S, de Bode S, Yudovin S, Shields WD, Vinters HV, Mathern GW
Neurology 2004 May 25;62(10):1712-21. doi: 10.1212/01.wnl.0000127109.14569.c3. PMID: 15159467

Prognosis

Innes EA, Marne FAL, Macintosh R, Nevin SM, Briggs NE, Vivekanandarajah S, Webster RI, Sachdev RK, Bye AME
Seizure 2024 Feb;115:1-13. Epub 2023 Dec 26 doi: 10.1016/j.seizure.2023.12.013. PMID: 38160512
Zhao S, Zhang X, Yang L, Wang Y, Jia S, Li X, Wang Z, Yang F, Liang M, Wang X, Wang D
J Hum Genet 2023 Sep;68(9):589-597. Epub 2023 May 10 doi: 10.1038/s10038-023-01145-1. PMID: 37161035
Kopciuch D, Barciszewska AM, Fliciński J, Zaprutko T, Kus K, Steinborn B, Nowakowska E
Acta Neurol Belg 2020 Feb;120(1):115-122. Epub 2019 Nov 13 doi: 10.1007/s13760-019-01236-x. PMID: 31721076
Spagnoli C, Falsaperla R, Deolmi M, Corsello G, Pisani F
Ital J Pediatr 2018 Nov 1;44(1):115. doi: 10.1186/s13052-018-0573-y. PMID: 30382869Free PMC Article
Lux AL
Acta Neurol Scand 2007 Apr;115(4 Suppl):37-44. doi: 10.1111/j.1600-0404.2007.00808.x. PMID: 17362275

Clinical prediction guides

Innes EA, Marne FAL, Macintosh R, Nevin SM, Briggs NE, Vivekanandarajah S, Webster RI, Sachdev RK, Bye AME
Seizure 2024 Feb;115:1-13. Epub 2023 Dec 26 doi: 10.1016/j.seizure.2023.12.013. PMID: 38160512
Szalai R, Melegh BI, Till A, Ripszam R, Csabi G, Acharya A, Schrauwen I, Leal SM, Komoly S, Kosztolanyi G, Hadzsiev K
Exp Mol Pathol 2020 Aug;115:104471. Epub 2020 May 21 doi: 10.1016/j.yexmp.2020.104471. PMID: 32446860
Takata A, Nakashima M, Saitsu H, Mizuguchi T, Mitsuhashi S, Takahashi Y, Okamoto N, Osaka H, Nakamura K, Tohyama J, Haginoya K, Takeshita S, Kuki I, Okanishi T, Goto T, Sasaki M, Sakai Y, Miyake N, Miyatake S, Tsuchida N, Iwama K, Minase G, Sekiguchi F, Fujita A, Imagawa E, Koshimizu E, Uchiyama Y, Hamanaka K, Ohba C, Itai T, Aoi H, Saida K, Sakaguchi T, Den K, Takahashi R, Ikeda H, Yamaguchi T, Tsukamoto K, Yoshitomi S, Oboshi T, Imai K, Kimizu T, Kobayashi Y, Kubota M, Kashii H, Baba S, Iai M, Kira R, Hara M, Ohta M, Miyata Y, Miyata R, Takanashi JI, Matsui J, Yokochi K, Shimono M, Amamoto M, Takayama R, Hirabayashi S, Aiba K, Matsumoto H, Nabatame S, Shiihara T, Kato M, Matsumoto N
Nat Commun 2019 Jun 7;10(1):2506. doi: 10.1038/s41467-019-10482-9. PMID: 31175295Free PMC Article
Thom M, Martinian L, Caboclo LO, McEvoy AW, Sisodiya SM
Acta Neuropathol 2008 Jun;115(6):697-700. Epub 2008 Jan 26 doi: 10.1007/s00401-008-0341-y. PMID: 18224329
Rahman MM, Akhter S, Karim BA
Mymensingh Med J 2004 Jan;13(1):67-70. PMID: 14747790

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