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FOXG1 syndrome

MedGen UID:
993083
Concept ID:
CN312528
Disease or Syndrome
Synonym: FOXG1-related epileptic-dyskinetic encephalopathy
 
Monarch Initiative: MONDO:0035383
Orphanet: ORPHA561854

Definition

A rare genetic neurological disorder characterized by early onset of microcephaly, severe global developmental delay and cognitive impairment, dyskinesia and hyperkinetic movements, visual impairment, autistic behavior, stereotypies, sleep disturbance, epilepsy, and cerebral malformations (such as corpus callosum hypogenesis, forebrain anomaly, and delayed myelination). Speech is minimal or absent, and ambulation is not attained. Patients with a larger 14q12 microdeletion show a more severe phenotype than those with intragenic alterations, with the addition of facial dysmorphism and agenesis of the corpus callosum. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFOXG1 syndrome

Professional guidelines

PubMed

Brimble E, Reyes KG, Kuhathaas K, Devinsky O, Ruzhnikov MRZ, Ortiz-Gonzalez XR, Scheffer I, Bahi-Buisson N, Olson H; FOXG1 Research Foundation
Orphanet J Rare Dis 2023 Jun 12;18(1):149. doi: 10.1186/s13023-023-02745-y. PMID: 37308910Free PMC Article
Akol I, Gather F, Vogel T
Int J Mol Sci 2022 Jan 16;23(2) doi: 10.3390/ijms23020954. PMID: 35055139Free PMC Article
Mitter D, Pringsheim M, Kaulisch M, Plümacher KS, Schröder S, Warthemann R, Abou Jamra R, Baethmann M, Bast T, Büttel HM, Cohen JS, Conover E, Courage C, Eger A, Fatemi A, Grebe TA, Hauser NS, Heinritz W, Helbig KL, Heruth M, Huhle D, Höft K, Karch S, Kluger G, Korenke GC, Lemke JR, Lutz RE, Patzer S, Prehl I, Hoertnagel K, Ramsey K, Rating T, Rieß A, Rohena L, Schimmel M, Westman R, Zech FM, Zoll B, Malzahn D, Zirn B, Brockmann K
Genet Med 2018 Jan;20(1):98-108. Epub 2017 Jun 29 doi: 10.1038/gim.2017.75. PMID: 28661489

Recent clinical studies

Etiology

Neul JL, Benke TA, Marsh ED, Suter B, Silveira L, Fu C, Peters SU, Percy AK; Rett syndrome Natural History Study Group
J Neurodev Disord 2023 Oct 13;15(1):33. doi: 10.1186/s11689-023-09502-z. PMID: 37833681Free PMC Article
Saby JN, Peters SU, Benke TA, Standridge SM, Swanson LC, Lieberman DN, Olson HE, Key AP, Percy AK, Neul JL, Nelson CA, Roberts TPL, Marsh ED
J Neurodev Disord 2023 Mar 4;15(1):10. doi: 10.1186/s11689-023-09479-9. PMID: 36870948Free PMC Article
Croci S, Carriero ML, Capitani K, Daga S, Donati F, Papa FT, Frullanti E, Lopergolo D, Lamacchia V, Tita R, Giliberti A, Benetti E, Niccheri F, Furini S, Lo Rizzo C, Conticello SG, Renieri A, Meloni I
Eur J Hum Genet 2020 Oct;28(10):1446-1458. Epub 2020 Jun 15 doi: 10.1038/s41431-020-0652-6. PMID: 32541681Free PMC Article
Mitter D, Pringsheim M, Kaulisch M, Plümacher KS, Schröder S, Warthemann R, Abou Jamra R, Baethmann M, Bast T, Büttel HM, Cohen JS, Conover E, Courage C, Eger A, Fatemi A, Grebe TA, Hauser NS, Heinritz W, Helbig KL, Heruth M, Huhle D, Höft K, Karch S, Kluger G, Korenke GC, Lemke JR, Lutz RE, Patzer S, Prehl I, Hoertnagel K, Ramsey K, Rating T, Rieß A, Rohena L, Schimmel M, Westman R, Zech FM, Zoll B, Malzahn D, Zirn B, Brockmann K
Genet Med 2018 Jan;20(1):98-108. Epub 2017 Jun 29 doi: 10.1038/gim.2017.75. PMID: 28661489
Cellini E, Vignoli A, Pisano T, Falchi M, Molinaro A, Accorsi P, Bontacchio A, Pinelli L, Giordano L, Guerrini R; FOXG1 Syndrome Study Group
Dev Med Child Neurol 2016 Jan;58(1):93-7. Epub 2015 Sep 6 doi: 10.1111/dmcn.12894. PMID: 26344814

Diagnosis

Neul JL, Benke TA, Marsh ED, Suter B, Silveira L, Fu C, Peters SU, Percy AK; Rett syndrome Natural History Study Group
J Neurodev Disord 2023 Oct 13;15(1):33. doi: 10.1186/s11689-023-09502-z. PMID: 37833681Free PMC Article
Saby JN, Peters SU, Benke TA, Standridge SM, Swanson LC, Lieberman DN, Olson HE, Key AP, Percy AK, Neul JL, Nelson CA, Roberts TPL, Marsh ED
J Neurodev Disord 2023 Mar 4;15(1):10. doi: 10.1186/s11689-023-09479-9. PMID: 36870948Free PMC Article
Hettige NC, Peng H, Wu H, Zhang X, Yerko V, Zhang Y, Jefri M, Soubannier V, Maussion G, Alsuwaidi S, Ni A, Rocha C, Krishnan J, McCarty V, Antonyan L, Schuppert A, Turecki G, Fon EA, Durcan TM, Ernst C
Stem Cell Reports 2022 Mar 8;17(3):475-488. Epub 2022 Feb 10 doi: 10.1016/j.stemcr.2022.01.010. PMID: 35148845Free PMC Article
Jang HN, Kim T, Jung AY, Lee BH, Yum MS, Ko TS
Medicine (Baltimore) 2021 Nov 24;100(47):e27949. doi: 10.1097/MD.0000000000027949. PMID: 34964776Free PMC Article
Mitter D, Pringsheim M, Kaulisch M, Plümacher KS, Schröder S, Warthemann R, Abou Jamra R, Baethmann M, Bast T, Büttel HM, Cohen JS, Conover E, Courage C, Eger A, Fatemi A, Grebe TA, Hauser NS, Heinritz W, Helbig KL, Heruth M, Huhle D, Höft K, Karch S, Kluger G, Korenke GC, Lemke JR, Lutz RE, Patzer S, Prehl I, Hoertnagel K, Ramsey K, Rating T, Rieß A, Rohena L, Schimmel M, Westman R, Zech FM, Zoll B, Malzahn D, Zirn B, Brockmann K
Genet Med 2018 Jan;20(1):98-108. Epub 2017 Jun 29 doi: 10.1038/gim.2017.75. PMID: 28661489

Therapy

Neul JL, Benke TA, Marsh ED, Suter B, Silveira L, Fu C, Peters SU, Percy AK; Rett syndrome Natural History Study Group
J Neurodev Disord 2023 Oct 13;15(1):33. doi: 10.1186/s11689-023-09502-z. PMID: 37833681Free PMC Article

Prognosis

Brimble E, Reyes KG, Kuhathaas K, Devinsky O, Ruzhnikov MRZ, Ortiz-Gonzalez XR, Scheffer I, Bahi-Buisson N, Olson H; FOXG1 Research Foundation
Orphanet J Rare Dis 2023 Jun 12;18(1):149. doi: 10.1186/s13023-023-02745-y. PMID: 37308910Free PMC Article
Ye XC, Roslin NM, Paterson AD, Lyons CJ, Pegado V, Richmond P, Shyr C, Fornes O, Han X, Higginson M, Ross CJ, Giaschi D, Gregory-Evans C, Patel MS, Wasserman WW
J Med Genet 2022 Jan;59(1):46-55. Epub 2020 Nov 30 doi: 10.1136/jmedgenet-2020-107226. PMID: 33257509Free PMC Article
Zepeda-Mendoza CJ, Bardon A, Kammin T, Harris DJ, Cox H, Redin C, Ordulu Z, Talkowski ME, Morton CC
Eur J Hum Genet 2018 Mar;26(3):374-381. Epub 2018 Jan 10 doi: 10.1038/s41431-017-0068-0. PMID: 29321672Free PMC Article
Mehrjouy MM, Fonseca ACS, Ehmke N, Paskulin G, Novelli A, Benedicenti F, Mencarelli MA, Renieri A, Busa T, Missirian C, Hansen C, Abe KT, Speck-Martins CE, Vianna-Morgante AM, Bak M, Tommerup N
Eur J Hum Genet 2018 Feb;26(2):186-196. Epub 2017 Dec 30 doi: 10.1038/s41431-017-0011-4. PMID: 29289958Free PMC Article

Clinical prediction guides

Brea-Fernández AJ, Souto-Trinei FA, Iglesias E, Caamaño P, Rodríguez Sánchez B, Gómez Lado C, Eiris J, Fernández-Prieto M, Barros F, Brea RJ, Carracedo Á
Cytogenet Genome Res 2023;163(5-6):301-306. Epub 2023 Dec 6 doi: 10.1159/000535660. PMID: 38056433
Brimble E, Reyes KG, Kuhathaas K, Devinsky O, Ruzhnikov MRZ, Ortiz-Gonzalez XR, Scheffer I, Bahi-Buisson N, Olson H; FOXG1 Research Foundation
Orphanet J Rare Dis 2023 Jun 12;18(1):149. doi: 10.1186/s13023-023-02745-y. PMID: 37308910Free PMC Article
Wong LC, Huang CH, Chou WY, Hsu CJ, Tsai WC, Lee WT
Autism Res 2023 May;16(5):953-966. Epub 2023 Mar 21 doi: 10.1002/aur.2916. PMID: 36942618
Bjerregaard VA, Levy AM, Batz MS, Salehi R, Hildonen M, Hammer TB, Møller RS, Desler C, Tümer Z
Genes (Basel) 2023 Jan 17;14(2) doi: 10.3390/genes14020246. PMID: 36833172Free PMC Article
Hettige NC, Peng H, Wu H, Zhang X, Yerko V, Zhang Y, Jefri M, Soubannier V, Maussion G, Alsuwaidi S, Ni A, Rocha C, Krishnan J, McCarty V, Antonyan L, Schuppert A, Turecki G, Fon EA, Durcan TM, Ernst C
Stem Cell Reports 2022 Mar 8;17(3):475-488. Epub 2022 Feb 10 doi: 10.1016/j.stemcr.2022.01.010. PMID: 35148845Free PMC Article

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