Short answer · Medically reviewed summary · Last updated: 2026-05-08
FOXG1 syndrome does not have a unique, dedicated ICD-10 or ICD-9 code; instead, it is clinically classified under broader categories for genetic disorders. In ICD-10, it is typically coded as G93.89 (Other specified disorders of brain) or Q04.8 (Other specified congenital malformations of brain), while in ICD-9, it was historically categorized under 742.8 (Other specified anomalies of brain). Why does FOXG1 syndrome lack a specific ICD code? Because FOXG1 syndrome is an ultra-rare neurodevelopmental disorder, it does not yet have a unique "chapter" in the International Classification of Diseases (ICD).
ICD10 code of FoxG1 Syndrome and ICD9 code
ICD-10 and ICD-9 codes for FoxG1 Syndrome, with classification details for clinicians, coders and patients.
FOXG1 syndrome does not have a unique, dedicated ICD-10 or ICD-9 code; instead, it is clinically classified under broader categories for genetic disorders. In ICD-10, it is typically coded as G93.89 (Other specified disorders of brain) or Q04.8 (Other specified congenital malformations of brain), while in ICD-9, it was historically categorized under 742.8 (Other specified anomalies of brain).
Why does FOXG1 syndrome lack a specific ICD code?
Because FOXG1 syndrome is an ultra-rare neurodevelopmental disorder, it does not yet have a unique "chapter" in the International Classification of Diseases (ICD). While the lack of a specific code can sometimes complicate insurance billing and administrative tracking, clinicians typically use the codes mentioned above to document the structural brain abnormalities and developmental delays associated with FOXG1 syndrome.
What are the primary clinical features of FOXG1 syndrome?
FOXG1 syndrome is caused by mutations or deletions of the FOXG1 gene, which is essential for brain development. Patients, including the 12 individuals currently connected through our DiseaseMaps community, often present with a distinct clinical profile:
- Severe intellectual disability and significantly delayed development.
- Impaired communication skills, often characterized by the absence of or very limited speech.
- Motor impairments, including difficulty walking or maintaining balance (ataxia).
- Common features such as microcephaly, epilepsy, and sleep disturbances.
- Specific movement disorders, often involving repetitive hand movements.
Is FOXG1 syndrome hereditary?
In the vast majority of cases, FOXG1 syndrome occurs as a de novo (new) mutation, meaning it is not inherited from either parent. A clinical geneticist can perform chromosomal microarray or gene sequencing to confirm the diagnosis. Genetic counseling is highly recommended for families to understand the recurrence risk, which is generally very low for future siblings.
Next steps
- Consult a pediatric neurologist or a clinical geneticist to confirm the diagnosis and discuss management.
- Connect with the FOXG1 syndrome community on DiseaseMaps.org to share experiences with other families.
- Register with the FOXG1 Research Foundation to stay updated on emerging clinical trials and therapeutic research.
Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment.
References
- NIH Genetic and Rare Diseases Information Center (GARD): FOXG1 syndrome.
- Orphanet: ORPHA254823 (FOXG1-related disorder).
- OMIM (Online Mendelian Inheritance in Man): Entry #164874.
- FOXG1 Research Foundation: Patient resources and clinical study information.
