Sturge-Weber syndrome with cerebellar involvement
Johns Hopkins Hospital, Baltimore, MD, USA.Journal of Neuroradiology (Impact Factor: 1.75). 09/2008; 36(1):57-60. DOI: 10.1016/j.neurad.2008.07.008
Sturge-Weber syndrome is a rare neurocutaneous disorder that typically presents with angiomas involving the face, ocular choroid and ipsilateral supratentorial leptomeninges. Posterior fossa involvement is extremely rare. We present two patients with simultaneous supra- and infratentorial involvement. Magnetic resonance imaging (MRI) and digital subtracted angiography (DSA) findings are discussed.
Conference Paper: Improved accuracy for the frameless stereotaxic operating microscope[Show abstract] [Hide abstract]
ABSTRACT: A frameless stereotaxic operating microscope for neurosurgery is discussed. The microscope projects computed tomography (CT) information into the microscope, superposing a tumor outline or guidance to predetermined targets in the surgical field in the correct scale, position, and orientation. The transposition of data from CT to OR (operating-room) space is accomplished with a set of fiducial points which are localized in both CT and OR space, eliminating the need for conventional mechanical stereotaxic frame attached to the head of the patient. Once this transformation matrix has been determined, the operating microscope's position is monitored and information derived from the CT scan is projected into the operating microscope. A nonimaging ultrasonic rangefinder, consisting of microphones and sound emitters, enables the precise location of the sound emitters in OR space to be determined. Under computer control, one sound emitter is fired, and the distance from it to each of the microphones is determined. The operating microscope has three sound emitters attached to it, so its position and orientation can be uniquely determined. Sound emitters are placed on the fiducial points on the patient's scalp, and the CT-OR transformation is determined. In this way, an open surgical field is maintained, yet stereotaxic information and guidance is available
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ABSTRACT: 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.
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ABSTRACT: The aim of this article was to describe the Sturge-Weber syndrome in a patient with orthodontic requirements. Pathologies involved in this syndrome affect facial cranial growth.
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