MRI with diffusion-weighted imaging in children and young adults with simultaneous supra- and infratentorial manifestations of Sturge-Weber syndrome.

Mark McIntosh,'s picture
PubMed URL:
Huisman TA
Author List: 
Arulrajah S
Ertan G
M Comi A
Tekes A
Lin DL
Huisman TA
J Neuroradiol
PubMed ID: 
Patients with Sturge-Weber syndrome (SWS) may present with neurological symptoms or neurocognitive deficits that cannot always be explained by the supratentorial findings seen on conventional MRI. Purpose of our study was to determine (a) what percentage of children with SWS have simultaneous supra- and infratentorial involvement and (b) what kind of infratentorial lesions are seen. In addition, we used DWI data to measure the ADC-values of normal appearing white matter (NAWM) to rule out impaired or delayed white matter maturation.Fifteen SWS patients who underwent MRI/DWI between January 2000 and August 2008 were studied. Images were retrospectively reviewed by two experienced pediatric neuroradiologists. ADC measurements of the NAWM were performed at multiple locations within the brain. ADC-values were compared with normative data and with 18 matched normal controls from our hospital.Infratentorial involvement was seen in six out of 15 patients (40%). Cerebellar lesions included leptomeningeal enhancement, atrophy and developmental venous anomaly. ADC-values were increased in the NAWM of the frontal, parietal and occipital lobes of both hemispheres and in the pons. The ADC-values of the cerebellar white matter were increased in six out of eight affected cerebellar lobes.Infratentorial involvement of SWS is more frequently than previously thought. ADC analysis of the NAWM reveals impaired/altered white matter maturation distant from the area of leptomeningeal angiomatosis. This may explain neurocognitive deficits. ADC analysis of the NAWM may serve as biomarker of tissue injury and therefore guide treatment options.
Published Date: 
March, 2010

Bradley L. Schlaggar, M.D., Ph.D., Named President and CEO of Kennedy Krieger Institute

We’re thrilled to welcome Bradley L. Schlaggar, M.D., Ph.D., to the Kennedy Krieger family as our next President and CEO.

Learn more.

Appointments & Referrals



Read inspiring stories, news and updates about the Institute's patient care, research, special education, professional training, and community programs.


Resource Finder


A free resource that provides access to information and support for individuals and families living with developmental disabilities.