Disease Directory Sanfilippo Syndrome
Metabolic

Sanfilippo Syndrome

Also known as: MPS III, mucopolysaccharidosis type III, heparan sulfate storage disease, Types A B C D

Prevalence

1-9 per 100,000 (Orphanet)

Onset

Infantile, Childhood

Type

Autosomal recessive genetic

Gene

SGSH (A), NAGLU (B), HGSNAT (C), GNS (D)

About Sanfilippo Syndrome

Sanfilippo syndrome (MPS III) is a group of four biochemically distinct lysosomal storage disorders (Types A-D) that all result in accumulation of heparan sulfate primarily in the brain, causing severe progressive neurodegeneration with relatively mild somatic involvement. Children typically develop normally for the first 1-3 years, then experience behavioral problems, hyperactivity, sleep disturbance, and relentless intellectual decline. No approved therapies exist, making clinical trials critical for this population.

Common Clinical Features

Hyperactivity and aggression Progressive intellectual decline Sleep disturbance Language regression Seizures Swallowing difficulties Coarse features (mild)

Clinical Trial Eligibility Tips

What to know before applying to Sanfilippo Syndrome trials.

Specify MPS III type (A, B, C, or D) — enzyme activity and gene mutations differ by type and trials are subtype-specific

CSF heparan sulfate level is the key CNS biomarker used in interventional trials for efficacy

Developmental staging and cognitive assessment scores are primary eligibility and outcome measures

Type A (SGSH) has the most active trial pipeline including gene therapy and enzyme replacement — enroll early before significant neurological decline

Patient Resources

Patient Organization

National MPS Society

Visit website ↗

Natural History Registry

Sanfilippo Research Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:581)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Sanfilippo Syndrome trials

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

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