Periventricular leukomalacia and cerebral palsy

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 (figure 1) 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 myleinated axons [nerves]) surrounding the ventricles.

Figure 1

How does this 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 (figure 2) and cerebellum contain nerves affecting motor control therefore babies with PVL may have a greater risk of developing cerebral palsy (cp).

Figure 2

What causes PVL?

It is 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 it diagnosed? What should I look for?

As PVL may lead to a diagnosis of cerebral palsy, the full effects and diagnosis will not necessarily be apparent until the child is older and developmental milestones such as sitting, crawling and walking have been reached. 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.

Seizures may be seen in children with PVL. A study in Israel (1) of 541 patients demonstrated that 18.7% of those experienced seizures. Seizures are more common in severe cases and those born prematurely and with low birth weight.

Infants with PVL often demonstrate inability to maintain a steady gaze and/or co-ordinate eye movements, and may have spontaneous rapid eye movements (nystagmus) or a squint (strabismus).

Your medical practitioner will monitor your child over a period of time and/or conduct a cranial ultrasound or MRI scan to detect any injury to brain tissue.

The prognosis of patients with PVL is dependent upon the severity and extent of white matter damage.

What treatments are available?

Currently there are no treatments for PVL. Patients are treated on an individual basis via close monitoring and treatments such as physiotherapy to treat specific effects.

Cerebral palsy

Infants with PVL may ultimately receive a diagnosis of cerebral palsy. The percentage varies but is generally reported as being between 20% - 60% (1) (2). This range is wide due to the variability of cerebral palsy. Despite varying grades of PVL and cp, children begin to exhibit signs of cp in a predictable manner for the condition. Another common outcome of PVL can be epilepsy but this may be due more to genetic and early environmental factors (3).

References
(1) Rezaie P, Dean A (2002)”Periventricular leukomalacia, inflammation and white matter lesions within the developing nervous system” Neuropathology 22:106-32. PMID 12416551.
(2) Fetters L, Huang H (2007), “Motor Development and sleep, play and feeding positions in very low birthweight infants with and without white matter disease” Developmental Medicine Child Neurology 49:807-13. PMID 17979857
(3) Gururaj AK, Sztriha L, Bener A. Dawodu A, Eapen V (2003). “Epilepsy in children with cerebral palsy.” Seizure 12:110-14. PMID 12566235

For more information on Scope

Contact Scope Response for information, advice and support on cerebral palsy and disability issues.

This information was last reviewed May 2010

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