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References in periodicals archive ?
Although the leptomeningeal angioma in SWS is typically a static lesion, it has been demonstrated by some to be of progressive nature.
The typical features of Sturge-Weber syndrome include port-wine facial nevus, associated with leptomeningeal angioma intracranial tram track calcification, and cerebral atrophy.
Isolated leptomeningeal angioma (Intracranial manifestations without facial lesions): No Glaucoma (Tortori_-Donati et al, 2005)
Neurological Involvement: The presence of leptomeningeal angiomas involving the pia mater is noted.
Sturge-Weber syndrome (SWS) is a rare sporadic disorder that occurs with a frequency of approximately 1/50 000 births [1, 2] and is characterized by cutaneous angioma in a trigeminal distribution, leptomeningeal angioma, and choroidal hemangioma [3].
The neurological signs are due to ipsilateral leptomeningeal angioma involving the occipital and posterior parietal lobes of the brain; vascular stasis with resultant ischemia leads to calcification and laminar cortical necrosis.
Type I: Both facial and leptomeningeal angiomas; may have glaucoma.
INTRODUCTION: Sturge-Weber syndrome is a sporadic neurocutaneous disease characterized by facial port-wine stain, ocular abnormalities (glaucoma and choroidal hemangioma) and leptomeningeal angioma. (1)
Type III--Isolated leptomeningeal angioma; usually no glaucoma.
The classical triad of Sturge-Weber syndrome is a port-wine stain on the face, glaucoma and leptomeningeal angiomas. While Type I might include the whole triad, Type II presents with the involvement of the skin without leptomeningeal angiomas.
The classic findings in the brain are unilateral or bilateral leptomeningeal angiomas (vascular malformations, not a tumor), hypoplastic cortical vessels, and an increase in size of the deep, draining venous structures.
Facial port-wine stains and associated leptomeningeal angiomas typify the vascular findings.