Disease Directory Aicardi Syndrome
Neurological

Aicardi Syndrome

Also known as: Corpus callosum agenesis with infantile spasms and chorioretinal lacunae, Aicardi-Goutieres overlap (distinct)

Prevalence

1-9 per 100,000 (Orphanet)

Onset

Infantile

Type

X-linked dominant (lethal in males, de novo in females)

Gene

Unknown (presumed X-linked, no gene confirmed)

About Aicardi Syndrome

Aicardi syndrome is a rare neurodevelopmental disorder occurring almost exclusively in females, characterized by the classic triad of corpus callosum agenesis (or other callosal malformations), infantile spasms, and chorioretinal lacunae (distinctive retinal lesions). Additional features include intellectual disability, vertebral and rib abnormalities, and choroid plexus papillomas. The causative gene has not been definitively identified; mutations in NEXMIF (KIAA2022) have been found in some patients.

Common Clinical Features

Corpus callosum agenesis Infantile spasms Chorioretinal lacunae Severe intellectual disability Vertebral anomalies Choroid plexus papillomas Absence of corpus callosum on MRI

Clinical Trial Eligibility Tips

What to know before applying to Aicardi Syndrome trials.

Classic triad (callosal agenesis, infantile spasms, chorioretinal lacunae) must be documented — ophthalmological confirmation of lacunae is critical

Brain MRI documentation of corpus callosum abnormality and ophthalmology report documenting lacunae are required at screening

No causative gene is confirmed — genetic panels are performed to exclude other conditions; results should be shared with trial coordinators

Natural history and observational studies are the primary research participation avenue given the lack of targeted therapies

Patient Resources

Patient Organization

Aicardi Syndrome Foundation

Visit website ↗

Natural History Registry

Aicardi Syndrome Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:50)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Aicardi Syndrome trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Aicardi Syndrome. Updated daily.

Related Rare Diseases