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FOXG1 syndrome

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Disease 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.

ORPHA:561854

Classification level: Disorder

Synonym(s):
  • FOXG1-related epileptic-dyskinetic encephalopathy

Source: PubMed ID 31454984

Prevalence: -

Inheritance: -

Age of onset: Neonatal

ICD-10: Q04.8

OMIM: 613454

UMLS: C3150705

GARD: 12825

A summary on this disease is available in Français, Español, Deutsch, Português, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2020.pdf) - Orphanet Urgences
Español (2020.pdf) - Orphanet Urgences
Clinical practice guidelines
Disease review articles
Clinical genetics review
English (2025) - GeneReviews
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