Pontine autosomal dominant microangiopathy with leukoencephalopathy

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ORPHA:477749OMIM:618564I67.8
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10Specialists8Treatment centers

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Overview

Pontine autosomal dominant microangiopathy with leukoencephalopathy, often called PADMAL, is a rare inherited disease that affects the small blood vessels in the brain. The word 'pontine' refers to the pons, a region at the base of the brain that controls important functions like balance, breathing, and movement. 'Microangiopathy' means disease of the small blood vessels, and 'leukoencephalopathy' means damage to the white matter — the parts of the brain that carry signals between different areas. Together, these changes disrupt how the brain works over time. PADMAL is caused by changes (mutations) in the COL4A1 or COL4A2 gene, which provide instructions for making a protein that supports the walls of small blood vessels. When these vessels are fragile or damaged, the brain does not get a steady, healthy blood supply. This leads to small strokes, bleeding in the brain, and progressive damage to white matter. Symptoms usually begin in adulthood and can include migraines, strokes at a young age, memory problems, difficulty walking, and mood changes. There is currently no cure for PADMAL. Treatment focuses on managing symptoms, reducing stroke risk, and supporting quality of life. This includes controlling blood pressure, avoiding medications that thin the blood too aggressively, and rehabilitation therapies. Because PADMAL is so rare and was only recently described as a distinct condition, research is still ongoing to better understand it and develop targeted treatments.

Also known as:

Key symptoms:

Recurrent migraines, often severeStroke or stroke-like episodes at a younger age than expectedMemory problems and difficulty thinking clearlyDifficulty walking or problems with balance and coordinationMood changes, depression, or personality changesWeakness or numbness on one side of the bodySlurred speechVision disturbancesFatigue and reduced energyGradual decline in daily functioning over time

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Pontine autosomal dominant microangiopathy with leukoencephalopathy.

View clinical trials →

No actively recruiting trials found for Pontine autosomal dominant microangiopathy with leukoencephalopathy at this time.

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Specialists

10 foundView all specialists →
GK
Gregor Kuhlenbäumer
Specialist
1 Pontine autosomal dominant microangiopathy with leukoencephalopathy publication
JR
Jessica Roos
SAN DIEGO, CA
Specialist
1 Pontine autosomal dominant microangiopathy with leukoencephalopathy publication
SM
Stefanie Müller
Specialist
1 Pontine autosomal dominant microangiopathy with leukoencephalopathy publication
AG
Anne Giese
SAINT PAUL, MN
Specialist
1 Pontine autosomal dominant microangiopathy with leukoencephalopathy publication
SA
Silke Appenzeller
Specialist
1 Pontine autosomal dominant microangiopathy with leukoencephalopathy publication
ER
Erich Bernd Ringelstein
Specialist
1 Pontine autosomal dominant microangiopathy with leukoencephalopathy publication
JF
Jens Fiehler
Specialist
1 Pontine autosomal dominant microangiopathy with leukoencephalopathy publication
KB
Klaus Berger
Specialist
1 Pontine autosomal dominant microangiopathy with leukoencephalopathy publication
CH
Christian Hagel
Specialist
1 Pontine autosomal dominant microangiopathy with leukoencephalopathy publication
AR
Arndt Rolfs
Specialist
1 Pontine autosomal dominant microangiopathy with leukoencephalopathy publication

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.Which gene mutation do I have, and what does that mean for my prognosis?,Should my children or siblings be tested for this condition?,What blood pressure target should I aim for, and how should I monitor it at home?,Are there any medications I should avoid because of my condition?,How often should I have brain MRI scans to monitor changes?,Are there any clinical trials I might be eligible for?,What rehabilitation services would help me most right now?

Common questions about Pontine autosomal dominant microangiopathy with leukoencephalopathy

What is Pontine autosomal dominant microangiopathy with leukoencephalopathy?

Pontine autosomal dominant microangiopathy with leukoencephalopathy, often called PADMAL, is a rare inherited disease that affects the small blood vessels in the brain. The word 'pontine' refers to the pons, a region at the base of the brain that controls important functions like balance, breathing, and movement. 'Microangiopathy' means disease of the small blood vessels, and 'leukoencephalopathy' means damage to the white matter — the parts of the brain that carry signals between different areas. Together, these changes disrupt how the brain works over time. PADMAL is caused by changes (muta

How is Pontine autosomal dominant microangiopathy with leukoencephalopathy inherited?

Pontine autosomal dominant microangiopathy with leukoencephalopathy follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Pontine autosomal dominant microangiopathy with leukoencephalopathy typically begin?

Typical onset of Pontine autosomal dominant microangiopathy with leukoencephalopathy is adult. Age of onset can vary across affected individuals.

Which specialists treat Pontine autosomal dominant microangiopathy with leukoencephalopathy?

10 specialists and care centers treating Pontine autosomal dominant microangiopathy with leukoencephalopathy are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.