Putting a Spotlight On
Each month The Arc of New Jersey Family Institute spotlights a different developmental disability to help build awareness and educate others about the disorder. Learn more below.
Periventricular Leukomalacia (PVL)
What is PVL?
Periventricular leukomalacia (PVL) is characterized by the death of the white matter of the brain due to softening of the brain tissue. It can affect fetuses or newborns; premature babies are at the greatest risk of the disorder. PVL is caused by a lack of oxygen or blood flow to the periventricular area of the brain, which results in the death or loss of brain tissue. The periventricular area-the area around the spaces in the brain called ventricles-contains nerve fibers that carry messages from the brain to the body's muscles.
What are the symptoms of PVL?
PVL may not be apparent until later months. Each baby may experience symptoms differently. The most common symptom of PVL is spastic diplegia, a form of cerebral palsy characterized by tight, contracted muscles, especially in the legs. The symptoms of PVL may resemble other conditions or medical problems
How is PVL diagnosed?
The healthcare provider will ask about your child’s symptoms and health history. He or she will give your child a physical exam. Your child may also have tests, such as:
Cranial ultrasound. This is a painless test that uses sound waves to make images of the tissues in the body. This test is used to view the baby's brain through the soft openings between the skull bones (the fontanelles). In a baby with PVL, the ultrasound shows cysts or hollow places in the brain tissue. Sometimes the condition can’t be seen with an ultrasound right away. So healthcare providers give babies at risk for PVL an ultrasound 4 to 8 weeks after birth.
MRI. This test uses large magnets, radio waves, and a computer to make images of the inside of the body. MRI may show some of the early changes in the brain tissue that occur with PVL.
How is PVL treated?
With PVL, the area of damaged brain tissue can affect the nerve cells that control motor movements. As the baby grows, the damaged nerve cells cause the muscles to become tight or shaky (spastic) and hard to move. Babies with PVL have a higher risk for cerebral palsy. This is a disorder that causes problems with muscle control. A child with PVL may also have thinking or learning problems.
There is no treatment to cure PVL. Babies at risk for PVL may need special care after discharge from the hospital. Follow-up may include physical therapy, occupational therapy, and speech therapy.
Resources:
https://www.urmc.rochester.edu/encyclopedia/content.aspx?contenttypeid=90&contentid=p02619
https://www.chop.edu/conditions-diseases/periventricular-leukomalacia-pvl
https://www.ninds.nih.gov/disorders/all-disorders/periventricular-leukomalacia-information-page
https://www.chop.edu/conditions-diseases/periventricular-leukomalacia-pvl
