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Revista Archivo Médico de Camagüey

versión On-line ISSN 1025-0255


PILA PEREZ, Rafael et al. Sturge-Weber syndrome: a case presentation. AMC [online]. 2010, vol.14, n.5. ISSN 1025-0255.

Background: Sturge-Weber syndrome is a very rare disease whose cardinal sign´s is the “port-wine stain". Objective: to present an unusual case of an eighty years old patient with a Sturge-Weber syndrome who has been admitted in several occasions for presenting severe ophthalmological and neurological alterations that warns to deepen in the study of these patients. The case was classified as a type I Sturge-Weber syndrome. Method: an eighty year-old male with “port-wine stain" antecedents from his birth, with epileptic seizure, almost constant cephalea, crisis of transient vascular ischemia in multiple occasions and admitted by ischemic encephalic vascular accident in two opportunities as a result of this, a residual left hemiplegia. Diagnosis is carried out by the clinic, computerized axial tomography, electroencephalogram and LCR characteristic. Attracting the attention he did not present a serious mental impairment. Results: important finding in this syndrome is the presence of “port-wine stain” with convulsions, glaucoma, cephalea and mental impairment. At present neuroimaging study is fundamental. Conclusions: under the presence of a “port-wine stain" in the region of the trigeminal nerve in childhood and youth forces to the patient's exhaustive study to achieve ophthalmological, neurological and cutaneous improvements with the current therapy and if wanted results are not achieved, to use surgery as mean of avoiding ophthalmologic and mental impairment that may reach to the loss of vision like it happened to our patient.


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