Disease Directory Niemann-Pick Disease
Metabolic

Niemann-Pick Disease

Also known as: NPA, NPB, NPC, Sphingomyelinase deficiency

Prevalence

1 in 120,000–150,000 (all types combined)

Onset

Variable (infancy to adulthood depending on type)

Type

Disease

Gene

SMPD1 (Types A and B), NPC1, NPC2 (Type C)

About Niemann-Pick Disease

Niemann-Pick disease encompasses several lysosomal storage disorders. Types A and B are caused by acid sphingomyelinase deficiency (SMPD1 mutations); Type A is severely neuronopathic with death in early childhood while Type B is a milder visceral form. Type C is caused by NPC1 or NPC2 mutations causing defective cholesterol trafficking and is characterised by progressive neurological decline. Arimoclomol is approved in the EU for NPC. Multiple clinical trials are active for NPC and for next-generation enzyme replacement for Types A/B.

Common Clinical Features

Enlarged liver and spleen Vertical gaze palsy (NPC hallmark) Progressive neurological deterioration Cerebellar ataxia Cognitive decline and dementia Seizures Pulmonary disease Gelastic cataplexy (NPC)

Clinical Trial Eligibility Tips

What to know before applying to Niemann-Pick Disease trials.

Vertical supranuclear gaze palsy is the clinical hallmark of NPC — if present, request NPC1/NPC2 gene sequencing and filipin staining urgently.

Plasma oxysterol biomarkers (24S-OHC, 25-OHC) are validated diagnostic and monitoring tools for NPC — ask your metabolic specialist about the NPC biomarker panel.

The National Niemann-Pick Disease Foundation maintains a patient contact database actively used by researchers — register before applying to trials.

Patient Resources

Patient Organization

National Niemann-Pick Disease Foundation

Visit website ↗

Natural History Registry

NPC Disease Registry

Join registry ↗

Orphanet

European reference resource for rare diseases

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Niemann-Pick Disease trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Niemann-Pick Disease. Updated daily.

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