Cerebral palsy

7 Periventricular leukomalacia (PVL)

What is periventricular leukomalacia (PVL)?

Periventricular leukomalacia (PVL) is damage and softening of the white matter within the brain near the cerebral ventricles.

  • Periventricular means around or near ventricles
  • Leuko means white
  • Malacia means softening

The ventricles within the brain are C-shaped spaces which produce cerebral spinal fluid (CSF). PVL is characterised by damage to the white matter (brain tissue consisting of myelinated axons (nerves) surrounding the ventricles.

How does PVL relate to cerebral palsy?

Like any damage to brain tissue, the exact location and depth of impairment will depend on how and where the body is affected. The areas around the ventricles near the basal ganglia and cerebellum contain nerves affecting movement. Babies with PVL may therefore have a greater risk of developing cerebral palsy.

What causes PVL?

It's hard to say. This area of the brain can be very susceptible to damage especially in premature or low birth weight babies where the tissue is very fragile. Possible causes can be lack of oxygen, ischemia (decreased blood flow), infection or rupturing of the uterus. Also hypotension (low blood pressure) resulting from foetal distress or caesarean birth can lead to decreased blood and oxygen supply to the developing brain and damage to the blood brain barrier which provides nutrients to the brain.

How is PVL diagnosed? 

As PVL may lead to a diagnosis of cerebral palsy, this may not be apparent until the child is older. The most common symptom is spastic diplegia (tight muscles in both limbs), contracted legs or difficulties in positioning when sleeping or feeding.

Severe PVL may be associated with quadriplegia. Children with PVL may have seizures. A study in Israel of 541 patients showed that 18.7% of those experienced seizures.

Seizures are more common in those born prematurely and with low birth weight. Infants with PVL often can't maintain a steady gaze or co-ordinate eye movements. They may have spontaneous rapid eye movements (nystagmus) or a squint (strabismus).

Your medical practitioner may conduct a cranial ultrasound or MRI scan to detect any injury to brain tissue. The prognosis of patients with PVL depends on the severity and extent of white matter damage.

What treatments are available for PVL?

Currently there are no specific treatments for PVL. Treatments such as physiotherapy can ease specific effects.

PVL and cerebral palsy

Infants with severe PVL may be more likely to receive a diagnosis of cerebral palsy as the severity of PVL is shown to be linked to major neurodevelopmental impairments. Another outcome of PVL can be epilepsy but this may be linked more to mild or moderate PVL or to genetic and early environmental factors.

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