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ISSN 0185-3252 (Print)
Revista de la Asociación Médica del Centro Médico ABC

2016, Number 2

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An Med Asoc Med Hosp ABC 2016; 61 (2)

Sturge-Weber syndrome associated with cortical dysplasia. A dual pathology case report

Suárez-Román AR, Ramírez-Navarrete E, Cruz-Martínez E, Romero-Figueroa JÁ

Language: Spanish
References: 11
Page: 132-136
PDF size: 722.21 Kb.


ABSTRACT

The Sturge-Weber syndrome is a rare congenital neurocutaneous disorder characterized by the association of facial, eye and brain capillary malformations. The most common neurological involvement is epilepsy, present in 75-90% of the patients. In refractory epilepsy, one of the causes is malformations in the cortical development. Brain MRI is the radiological method to display the malformed brain area, and electroencephalogram is used for the precise location of the epileptogenic focus. We present the case of a one-year, 11-month-old female with clinical and imaging evidence of left-side Sturge-Weber and electroencephalography evidence most consistent with probable right-side cortical dysplasia. We address the clinical description, paraclinical studies, and the outcome of our multidisciplinary approach. Our patient presents two independent diseases whose association has not been previously described in the literature. Although the incidence of seizure is high in Sturge-Weber, our case has evidence of seizure onset in the contralateral hemisphere.


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