Periventrikulär leukomalaci
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Children may also experience cognitive delays, learning difficulties, or problems with vision. White matter consists of nerve fibers, called axons, coated with a fatty substance called myelin, giving it a white appearance.
The main function of white matter is to transmit electrical signals and information between different parts of the brain and between the brain and the spinal cord.
PVL may manifest as cerebral palsy (>50% in the setting of cystic PVL), intellectual disability, or visual disturbance. The disorder is diagnosed with an ultrasound of the head.
There is no specific treatment for PVL. Treatment is symptomatic and supportive. Magnetic resonance imaging (MRI) provides more detailed images of the brain’s white matter.
Physical therapy improves motor skills, reduces muscle stiffness, and enhances coordination and balance.
It is important to note that both periventricular and subcortical leukomalacia correspond to a continuous disease spectrum.
All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.
Motor impairments are common, with PVL being a leading cause of cerebral palsy, particularly spastic diplegia, which affects muscle tone and movement, especially in the legs. Sonography is sensitive for the detection of hemorrhage, periventricular leukomalacia, and hydrocephalus.
On ultrasound, hyperechoic areas are firstly identified in a distinctive fashion in the periventricular area, more often at the peritrigonal area and in an area anterior and lateral to the frontal horns (periventricular white matter should be less echogenic than the choroid plexus).
These are watershed areas that are sensitive to ischemic injury.
Please refer to the article on patterns of neonatal hypoxic-ischemic brain injury for a relation between the perinatal brain maturation process and these lesions.
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PVL is most common in premature neonates (less than 34 weeks' gestational age, with a median gestational age of 30 weeks) and <1500 grams at birth.
Common early indicators include developmental delays, such as difficulties with head control, sitting, or crawling. doi:10.1148/rg.261055033 - Pubmed
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It likely occurs as a result of hypoxic-ischemic lesions caused by impaired perfusion in watershed areas, which in premature infants are periventricular.
Epelman M, Daneman A, Blaser S et al. Differential Diagnosis of Intracranial Cystic Lesions at Head US: Correlation with CT and MR Imaging. The disorder is caused by a lack of oxygen or blood flow to the periventricular area of the brain. They can help connect patients with new and upcoming treatment options.
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How can I or my loved one help improve care for people with periventricular leukomalacia?
Consider participating in a clinical trial so clinicians and scientists can learn more about PVL.
Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease. Other contributing factors include infections in the womb or shortly after birth, premature rupture of membranes, or bleeding within the brain (intraventricular hemorrhage), all of which can lead to oxygen deprivation or reduced blood supply, damaging the white matter.
Signs of PVL may not be obvious at birth, becoming apparent as the child grows.
PVL can affect fetuses or newborns, although premature babies are at greatest risk. It is likely that infection or vasculitis also plays a role in pathogenesis.