[Sturge-Weber syndrome: experience with 14 cases].

OBJECTIVE

The objective of this study was to review the cases of Sturge-Weber syndrome (SWS) diagnosed and followed-up in our center over the last 25 year period in order to evaluate their clinical characteristics, evolution and therapeutical response.

METHODS

A retrospective review of the records of patients diagnosed of SSW (facial nevus flammeus at least over the first branch of the trigeminal nerve and ipsilateral leptomeningeal angioma) was performed.

RESULTS

Fifteen patients were found in our records, with one of them being excluded from the study due to the lack of follow-up. From the remaining 14, 11 had been diagnosed before the age of 18 months. Nevus flammeus was located on the right side in 8 cases, bilaterally in 3 and on the left side in the other 3 patients. Four cases had glaucoma, 3 of which had good evolution after trabeculotomy. Thirteen of the 14 patients had seizures, usually contralateral to the nevus flammeus. The main drugs used were phenytoin, phenobarbital, valproic acid and carbamazepine. Ten of the 13 patients treated had a good therapeutical response. The EEG was abnormal in 12 patients, two of them occurring during the follow-up period. The main abnormality found was an interhemispherical asymmetry. Nine patients developed hemiparesis. In reference to the neuroimaging. MR was better than CT in evaluating parenchymatous atrophy, which was present in all patients, and the abnormalities of the white and grey matters (9 and 8 patients, respectively).

CONCLUSIONS

Early evaluation of newborns with nevus flammeus affecting the first branch of the trigeminal nerve must be done, including an MR with contrast and an ophthalmological exam.