[Sturge-Weber syndrome. Early manifestation and visualization of disease course].

BACKGROUND

The purpose of this study was to evaluate ultrasound, MRI, and CT investigations in children with Sturge-Weber syndrome.

PATIENTS AND METHODS

From 1996 to 2005, a total of 15 investigations of the brain performed in 6 children with Sturge-Weber syndrome were reviewed. We evaluated four ultrasound, five CT, and six MRI exams.

RESULTS

With ultrasound an increase of the echogenicity of the periventricular white matter was depicted in the first days of life. MRI is the best modality to demonstrate the vascular malformation, the impaired cerebral venous drainage, and the atrophy of one hemisphere.

CONCLUSIONS

One-sided periventricular hyperechogenicity on ultrasound can be an early sign of Sturge-Weber syndrome in children with a nevus flammeus. MRI is the method of choice to diagnose Sturge-Weber syndrome and to follow up these children if the neurological status of the patients changes.