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This chapter presents the clinical features, etiology, and treatment of Sturge-Weber syndrome (SWS). SWS is a rare, sporadic, congenital disorder arising from an early developmental lesion affecting the facial skin, the eye, and the central nervous system. The association of neurological and developmental deterioration and the onset of seizures means that patients with SWS often suffer considerable disability. The effect of choroidal or ciliary body hemangioma in SWS interferes with the angle of the eye, causing elevated episcleral venous pressure or hypersecretion of fluid. Epileptic seizures are the predominant symptom of SWS and occur in about 80% of cases presenting port-wine stain (PWS) and leptomeningeal angioma. Magnetic resonance imaging (MRI) should be performed at a distance of a seizure event to avoid false interpretation of gadolinium enhancement, due to contrast leakage because of alteration of the blood-brain barrier.
This chapter talks about the neurocutaneous syndromes such as hypomelanosis of Ito (HI), incontinentia pigmenti (IP), nevus sebaceous (NS) syndrome and unilateral somatic intracranial hypoplasia. Chromosomal mosaicism is recognized as the pathogenic basis of many cases of HI and related disorders. It can explain the protean clinical manifestations of this condition and their often asymmetrical expression. Cerebral lesions of IP patients commonly extend radially through cortical and subcortical zones, involving cortex, subcortical and deep white matter, ependymal and subependymal zones of one or both cerebral hemispheres. Epilepsy usually appears after a variable period of evolution when the subcortical lesions are apparent in the cerebral hemisphere ipsilateral to the facial hemiatrophy. Unilateral hypoplasia of a polymicrogyric cerebral hemisphere, of the brainstem, cerebellum, and of the intracranial arteries on the same side and a hypoplastic hemibody commonly occur.
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