Disease Directory Krabbe Disease
Metabolic

Krabbe Disease

Also known as: Globoid cell leukodystrophy, galactosylceramide lipidosis, GALC deficiency

Prevalence

1-9 per 100,000 (Orphanet)

Onset

Infantile, Juvenile, Adult

Type

Autosomal recessive genetic

Gene

GALC

About Krabbe Disease

Krabbe disease is a rapidly progressive and often fatal lysosomal storage disorder caused by mutations in the GALC gene encoding galactocerebrosidase. Deficiency of this enzyme leads to accumulation of psychosine, a toxic lipid that destroys the myelin sheath protecting nerve cells. The early infantile form is most common and most severe, presenting before 6 months of age with irritability, feeding difficulties, and rapid neurological decline.

Common Clinical Features

Irritability Hypertonia Peripheral neuropathy Optic atrophy Seizures Developmental regression Hyperthermia

Clinical Trial Eligibility Tips

What to know before applying to Krabbe Disease trials.

Hematopoietic stem cell transplantation (HSCT) eligibility is age and symptom-severity dependent — pre-symptomatic newborns have the best outcomes

Newborn screening status is often required documentation for early infantile trial enrollment

GALC enzyme activity below a threshold percentage of normal is a standard inclusion criterion

Gene therapy trials typically exclude patients who have already received HSCT

Patient Resources

Patient Organization

Hunter's Hope Foundation

Visit website ↗

Natural History Registry

Krabbe Disease Natural History Study (NIH)

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:487)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Krabbe Disease trials

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

Related Rare Diseases