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Título

STURGE WEBER SYNDROME IN ADULT: A CASE REPORT

RESUMO

CASE PRESENTATION:

Patient FB, 41 years after entering the service complaining of left hemiparesis (strength grade 4-). The same refers that since childhood (5 months - 5 years) he presents recurrent episodes of reported deficit, which improve with the use of phenytoin, causing delay in his motor development. He denies other comorbidities or neurological pathologies in the family. In addition to the deficit, diagnosed as a focal crisis, a cutaneous vascular malformation on the right hemiface, compatible with a flaming nevus, called attention on physical examination. Due to this clinical finding and previous history, a brain resonance imaging (MRI) was performed, which showed volumetric reduction in the right parietal hemisphere and cortical leptomeningeal uptake compatible with anomaly of the cortical venous system, prominence of varicose leptomeningeal veins and prominence of the brain-related choroid plexus. trigeminal angiomatous.
The seizures were controlled with phenytoin, a drug that the patient received when he was discharged, without any focal deficits. Scheduled outpatient follow-up for seizure control and follow-up.

DISCUSSION:

Sturge Weber syndrome is a rare condition with an incidence of 1/50,000 to 1/230,000 live births. It is explained by the abnormal persistence of the embryonic vascular system which is located around the cephalic region of the neural tube. Its most common clinical presentation is the vascular dermato alteration manifested in the face, in addition to seizures. Less presented per patient, in addition to classic change seen on MRI of the skull.


FINAL COMMENTS:
The management of patients with this syndrome shows challenges due to its rarity and general medical ignorance about it. Its treatment aims to control epilepsy and decrease visual deficits. In view of the benefit of the correct treatment, it is important to disseminate knowledge about this pathology in the medical and academic environment, prioritizing investigation with an image exam which demonstrates lesions characteristic of the disease.

Palavras Chave

Stuge-werber, convulsive crises, seizures

Área

Neurogenética