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- W156716035 abstract "Publisher Summary This chapter describes various aspects of the clinical experience with the Sturge–Weber syndrome (SWS). The clinical presentation of the SWS is variable, depending on the extent of pial angiomatosis, and age of onset of neurologic and other deficits. SWS belongs to the group of neurocutaneous disorders known as the phakomatoses. Both the nervous system and the integumentary system are derived from ectoderm, embryologically, though blood vessels are mesenchymal in origin. The most sophisticated functional neuro-imaging methodology is provided by the technique of positron-emission tomography (PET), based on the emission of gamma radiation perpendicular to the path of collision of electrons with positrons in cerebral tissue. In cases of the SWS, where a cortical lesion has been identified by neuroimaging, such as MRI, and the seizures have been pharmacologically resistant to major antiepileptic drugs (AEDs), undue delay should be avoided to prevent the development of unacceptable neurological deficits. The extent of cortical involvement may play a role in the severity of the clinical presentation and the choice of surgical management of children with SWS." @default.
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- W156716035 date "1999-01-01" @default.
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- W156716035 title "Sturge-Weber Syndrome" @default.
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- W156716035 doi "https://doi.org/10.1016/b978-012422150-5/50009-3" @default.
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