Sturge-Weber syndrome and epilepsy: An argument for aggressive seizure management in these patients

Department of Neurology, Kennedy Krieger Institute & Johns Hopkins Medicine, Baltimore, MD, USA.
Expert Review of Neurotherapeutics (Impact Factor: 2.78). 09/2007; 7(8):951-6. DOI: 10.1586/14737175.7.8.951
Source: PubMed


Sturge-Weber syndrome (SWS) involves vascular malformations of the skin (facial port-wine stain), eye (glaucoma) and the brain (leptomeningeal angioma). Children born with a port-wine stain on the upper part of the face are also at risk for brain involvement. These infants and young children often develop seizures and other neurologic impairments. Progression in neurologic deficits does occur in some patients, but this is quite variable. A diagnosis of brain involvement is made with head computed tomography and contrast-enhanced MRI, but the sensitivity of standard imaging in young asymptomatic infants is low. Seizures occur in more than 75% of affected individuals. Clinical course and functional imaging suggest a role for both cerebral perfusion impairments and seizures in the development of neurologic deficits. Several controversies exist in the management of seizures and other neurologic impairments in SWS. Continued efforts are needed to develop a multicentered network for SWS clinical trials. Future research should be focused on this goal and on studies to improve our understanding of the cause(s) and molecular neuropathology of SWS.

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    • "This finding may partly explain the new or increased neurologic deficits after seizures in patients with Sturge-Weber syndrome. Recent positron-emission tomography imaging studies, and our clinical experience, suggest that prolonged freedom from seizures is associated with improved cerebral glucose metabolism in affected regions and improved developmental progress [3]. Therefore, it seems reasonable Table 1. "
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    ABSTRACT: The pathophysiology of Sturge-Weber syndrome is poorly understood, and ear, nose, and throat involvement is possible. These issues can result in frequent illnesses or airway obstruction, affecting patients' neurologic status. Patients with definite brain involvement who reported potential ear, nose, and throat issues on intake questionnaires underwent retrospective reviews of their medical records. We examined the relationships between these issues, secondary surgical interventions, and patients' neurologic status. The most common complaints involved the sinuses and frequent ear infections. Six patients underwent placement of ear tubes, leading to improvements in migraines and stroke-like episodes in one patient, and improved seizure control in four others. Obstructive sleep apnea was confirmed in three patients who underwent sleep studies. Tonsil or adenoid removal occurred in another three patients. Surgery resulted in marked improvements regarding excessive drooling, daytime sleepiness, and breathing problems. These findings suggest that ear, nose, and throat problems occur frequently in patients with Sturge-Weber Syndrome, and when repeated ear infections are associated with uncontrolled seizures, early placement of ear tubes may be beneficial. Furthermore, patients with facial tissue hypertrophy may be at risk for obstructive sleep apnea, and should be appropriately evaluated.
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    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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