Disease Directory Canavan Disease
Neurological

Canavan Disease

Also known as: Aspartoacylase deficiency, ASPA deficiency, spongy degeneration of the brain, van Bogaert-Bertrand disease

Prevalence

1-9 per 100,000 (Orphanet, higher in Ashkenazi Jewish population)

Onset

Infantile

Type

Autosomal recessive genetic

Gene

ASPA

About Canavan Disease

Canavan disease is a fatal leukodystrophy caused by deficiency of aspartoacylase (ASPA), which metabolizes N-acetylaspartate (NAA) in the brain. Accumulation of NAA in brain cells disrupts myelin formation and maintenance, causing progressive spongy degeneration of the white matter. Affected children appear normal at birth but develop macrocephaly, hypotonia, head lag, and severe intellectual disability by 3-6 months, with a fatal course usually in childhood.

Common Clinical Features

Macrocephaly Hypotonia and head lag Severe intellectual disability Developmental regression Optic atrophy Seizures White matter spongy degeneration on MRI

Clinical Trial Eligibility Tips

What to know before applying to Canavan Disease trials.

Urine N-acetylaspartate (NAA) level markedly elevated is the pathognomonic biomarker required for trial enrollment

ASPA enzyme activity in fibroblasts or leukocytes and biallelic ASPA variants are required for genetic confirmation

Gene therapy trials (AAV-based) are the primary interventional pathway — no prior gene therapy is a universal exclusion

Brain MRI pattern (diffuse white matter abnormality with specific spectroscopy findings) must be documented at baseline

Patient Resources

Patient Organization

Canavan Foundation

Visit website ↗

Orphanet

European reference resource for rare diseases (ORPHA:141)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Canavan Disease trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Canavan Disease. Updated daily.

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