Periventricular leukomalacia

 

Periventricular leukomalacia (PVL) is characterized by the death of the white matter near the cerebral ventricles due to softening of the brain tissue. It can affect fetuses or newborns; premature babies are at the greatest risk of the disorder.

Causes: 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 within the brain

Presentation: Although babies with PVL generally have no outward signs or symptoms of the disorder, they are at risk for motor disorders (especially of the lower limbs), delayed mental development, coordination problems, and vision and hearing impairments. Children with PVL have higher levels of nystagmus, strabismus, optic nerve hypoplasia and refractive error. PVL may be accompanied by a hemorrhage or bleeding in the periventricular-intraventricular area (the area around and inside the ventricles), and can lead to cerebral palsy.

Diagnosis: The disorder can be diagnosed by ultrasound, but magnetic resonance imaging of the head is diagnostically more accurate.

Treatment:    The clinically available anticonvulsant topiramate, when administered post-insult in vivo, is considered to be protective against selective hypoxicischemic white matter injury and decreases the subsequent neuromotor deficits. Topiramate also attenuates AMPAkainate receptor-mediated cell death and calcium influx, as well as kainate-evoked currents in developing oligodendrocytes.[1]

Children with PVL should receive regular medical screenings to determine appropriate interventions.

Prognosis: The prognosis for individuals with PVL depends upon the severity of the brain damage. Some children exhibit fairly mild symptoms, while others have significant deficits and disabilities.