Disease Directory Pelizaeus-Merzbacher Disease
Neurological

Pelizaeus-Merzbacher Disease

Also known as: PMD, PLP1 mutation, hypomyelinating leukodystrophy 1, connatal and classic Pelizaeus-Merzbacher

Prevalence

1-9 per 100,000 (Orphanet)

Onset

Neonatal, Infantile

Type

X-linked genetic

Gene

PLP1

About Pelizaeus-Merzbacher Disease

Pelizaeus-Merzbacher disease (PMD) is an X-linked hypomyelinating leukodystrophy caused by mutations in PLP1, encoding proteolipid protein 1, a major component of myelin in the central nervous system. Inadequate or abnormal myelination causes progressive neurological impairment including nystagmus, hypotonia, ataxia, spasticity, and intellectual disability. The connatal form is most severe with onset at birth; the classic form presents in early infancy. There are no approved therapies; stem cell and gene therapy approaches are in active development.

Common Clinical Features

Nystagmus (early onset) Hypotonia Stridor Ataxia Spasticity Intellectual disability Seizures

Clinical Trial Eligibility Tips

What to know before applying to Pelizaeus-Merzbacher Disease trials.

PLP1 mutation type (duplication, point mutation, deletion, null mutation) determines phenotype and is a critical eligibility factor for gene therapy trials

Brain MRI showing hypomyelination pattern is required for diagnostic confirmation and trial documentation

Allogeneic stem cell transplantation has been attempted in small studies — transplant status may affect future trial eligibility

PLP1 null mutations causing a distinct milder spastic paraplegia form (SPG2) may be excluded from PMD-specific trials

Patient Resources

Patient Organization

United Leukodystrophy Foundation

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Natural History Registry

Global Leukodystrophy Initiative (GLIA)

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Orphanet

European reference resource for rare diseases (ORPHA:702)

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NORD

National Organization for Rare Disorders

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Find recruiting Pelizaeus-Merzbacher Disease trials

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

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