U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

GNAO1-related developmental delay-seizures-movement disorder spectrum

MedGen UID:
1830103
Concept ID:
C5680303
Disease or Syndrome
Synonyms: G protein subunit alpha o1-related developmental delay, seizures, movement disorder spectrum; GNAO1-related developmental delay, seizures, movement disorder spectrum; GNAO1-related spectrum
SNOMED CT: GNAO1-related developmental delay, seizures, movement disorder spectrum (1281842000); GNAO1-related spectrum (1281842000); G protein subunit alpha o1-related developmental delay, seizures, movement disorder spectrum (1281842000)
 
Monarch Initiative: MONDO:0035660
Orphanet: ORPHA592564

Definition

A rare genetic neurological disorder with a phenotypic spectrum of mild to severe developmental delay and hypotonia variably associated with intellectual disability, early-onset seizures and movement disorders, such as dystonia, ataxia, chorea and dyskinesia. Brain imaging may show delayed myelination, thin corpus callosum or cerebral atrophy. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • GNAO1-related developmental delay-seizures-movement disorder spectrum

Recent clinical studies

Diagnosis

Novelli M, Galosi S, Zorzi G, Martinelli S, Capuano A, Nardecchia F, Granata T, Pollini L, Di Rocco M, Marras CE, Nardocci N, Leuzzi V
Parkinsonism Relat Disord 2023 Jun;111:105405. Epub 2023 Apr 29 doi: 10.1016/j.parkreldis.2023.105405. PMID: 37142469
Gambardella ML, Pede E, Orazi L, Leone S, Quintiliani M, Amorelli GM, Petrianni M, Galanti M, Amore F, Musto E, Perulli M, Contaldo I, Veredice C, Mercuri EM, Battaglia DI, Ricci D
Genes (Basel) 2023 Feb 22;14(3) doi: 10.3390/genes14030544. PMID: 36980817Free PMC Article
Wirth T, Garone G, Kurian MA, Piton A, Millan F, Telegrafi A, Drouot N, Rudolf G, Chelly J, Marks W, Burglen L, Demailly D, Coubes P, Castro-Jimenez M, Joriot S, Ghoumid J, Belin J, Faucheux JM, Blumkin L, Hull M, Parnes M, Ravelli C, Poulen G, Calmels N, Nemeth AH, Smith M, Barnicoat A, Ewenczyk C, Méneret A, Roze E, Keren B, Mignot C, Beroud C, Acosta F Jr, Nowak C, Wilson WG, Steel D, Capuano A, Vidailhet M, Lin JP, Tranchant C, Cif L, Doummar D, Anheim M
Mov Disord 2022 Jul;37(7):1547-1554. Epub 2022 Jun 20 doi: 10.1002/mds.29074. PMID: 35722775Free PMC Article
Axeen E, Bell E, Robichaux Viehoever A, Schreiber JM, Sidiropoulos C, Goodkin HP
Pediatr Neurol 2021 Aug;121:28-32. Epub 2021 May 12 doi: 10.1016/j.pediatrneurol.2021.05.005. PMID: 34139551

Prognosis

Novelli M, Galosi S, Zorzi G, Martinelli S, Capuano A, Nardecchia F, Granata T, Pollini L, Di Rocco M, Marras CE, Nardocci N, Leuzzi V
Parkinsonism Relat Disord 2023 Jun;111:105405. Epub 2023 Apr 29 doi: 10.1016/j.parkreldis.2023.105405. PMID: 37142469

Clinical prediction guides

Wirth T, Garone G, Kurian MA, Piton A, Millan F, Telegrafi A, Drouot N, Rudolf G, Chelly J, Marks W, Burglen L, Demailly D, Coubes P, Castro-Jimenez M, Joriot S, Ghoumid J, Belin J, Faucheux JM, Blumkin L, Hull M, Parnes M, Ravelli C, Poulen G, Calmels N, Nemeth AH, Smith M, Barnicoat A, Ewenczyk C, Méneret A, Roze E, Keren B, Mignot C, Beroud C, Acosta F Jr, Nowak C, Wilson WG, Steel D, Capuano A, Vidailhet M, Lin JP, Tranchant C, Cif L, Doummar D, Anheim M
Mov Disord 2022 Jul;37(7):1547-1554. Epub 2022 Jun 20 doi: 10.1002/mds.29074. PMID: 35722775Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...