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- W1983199105 abstract "El síndrome de Sturge-Weber es un síndrome neurocutáneo raro cuya gravedad está determinada por el grado de afectación cerebral y el control de la epilepsia. Los autores describen y analizan las características clínicas y de neuroimagen de este síndrome, a través de un estudio retrospectivo de 13 pacientes (8 niñas; con edad en la primera consulta entre los 15 días y los 9 años). Doce presentaban angioma facial y una atriquia en la zona de afectación cerebral. Se diagnosticó epilepsia en 6 casos, hemiplejía en 4, retraso psicomotor en 7 y glaucoma en 4 casos. Se encontraron alteraciones cerebrales en 10 niños, 3 sin sintomatología neurológica. La clínica presentada es variable y no siempre hay relación entre la gravedad clínica y las anomalías de neuroimagen, que pueden aparecer hasta en ausencia de síntomas neurológicos. Sturge Weber Syndrome is a rare neurocutaneous syndrome in which the severity is determined by degree of brain involvement and control of epilepsy. The authors describe and analyse clinical and imaging features of this syndrome, through a retrospective study of 13 patients (8 girls; aged between 15 days and 9 years at first visit). Twelve had facial angioma and one had atrichia corresponding to the area of brain involvement. Epilepsy was diagnosed in 6 cases, hemiplegia in 4, psychomotor delay in 7, and glaucoma in 4. Cerebral abnormalities were found in 10 children, 3 without neurological symptoms. The clinical signs and symptoms vary and there is not always a relationship between the severity of the clinical and neuroimaging abnormalities, which may occur even in the absence of neurological symptoms." @default.
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- W1983199105 date "2012-12-01" @default.
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- W1983199105 title "Síndrome de Sturge-Weber: variabilidad clínica y de neuroimagen" @default.
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- W1983199105 doi "https://doi.org/10.1016/j.anpedi.2012.03.004" @default.
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