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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

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
Słowińska M, Kotulska K, Szymańska S, Roberds SL, Fladrowski C, Jóźwiak S
Pediatr Neurol 2021 Feb;115:21-27. Epub 2020 Nov 5 doi: 10.1016/j.pediatrneurol.2020.11.003. PMID: 33310533
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
Berry-Kravis E, Raspa M, Loggin-Hester L, Bishop E, Holiday D, Bailey DB
Am J Intellect Dev Disabil 2010 Nov;115(6):461-72. doi: 10.1352/1944-7558-115.6.461. PMID: 20945999

Prognosis

Jose M, Fasaludeen A, Pavuluri H, Rudrabhatla PK, Chandrasekharan SV, Jose J, Banerjee M, Sundaram S, Radhakrishnan A, Menon RN
Seizure 2024 Feb;115:20-27. Epub 2024 Jan 3 doi: 10.1016/j.seizure.2023.12.017. PMID: 38183824
Smith AW, Gutierrez-Colina AM, Roemisch E, Hater B, Combs A, Shoulberg AM, Modi AC
Epilepsy Behav 2021 Feb;115:107718. Epub 2021 Jan 10 doi: 10.1016/j.yebeh.2020.107718. PMID: 33440273
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
Ramey WL, Martirosyan NL, Lieu CM, Hasham HA, Lemole GM Jr, Weinand ME
Clin Neurol Neurosurg 2013 Dec;115(12):2411-8. Epub 2013 Oct 11 doi: 10.1016/j.clineuro.2013.09.035. PMID: 24169149
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

Saini L, Mukherjee S, Gunasekaran PK, Malhi P, Saini AG, Sharma R, Sharawat IK, Suthar R, Sahu JK, Sankhyan N
Neuropediatrics 2023 Oct;54(5):335-338. Epub 2023 Jun 28 doi: 10.1055/s-0043-1770937. PMID: 37379859
Smith AW, Gutierrez-Colina AM, Roemisch E, Hater B, Combs A, Shoulberg AM, Modi AC
Epilepsy Behav 2021 Feb;115:107718. Epub 2021 Jan 10 doi: 10.1016/j.yebeh.2020.107718. PMID: 33440273
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
Briggs SW, Mowrey W, Hall CB, Galanopoulou AS
Epilepsia 2014 Jan;55(1):94-102. Epub 2013 Oct 28 doi: 10.1111/epi.12424. PMID: 24321005Free PMC Article
Freitag H, Tuxhorn I
Epilepsia 2005 Apr;46(4):561-7. doi: 10.1111/j.0013-9580.2005.03504.x. PMID: 15816951

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