Abstracts

Introduction: In Sturge Weber syndrome, epilepsy is a common feature. Seizures occur in 75-90% of cases that constitute the most disabling neurological symptoms. In the world literature reported age of onset varies from 2 months to 14 years, being more frequent before the year of age. The leptomenigeal angyomatoses is more frequent in the occipital region therefore the seizures more frequently are partial motor features limited to a body part or limb. Have also been observed apparently generalized seizures and infantile spasms typically may precede other seizures. One of the features in this syndrome is the experience in long-term crisis or unilateral epileptic status. The development is not favorable in the presence of epilepsy and intellectual and neurological function. Some points of poor prognosis indicate that early seizures are prone to generalization, increased frequency, increased time post-ictal state, progressive and rapid deterioration and evidence of cerebral atrophy in the area of injury. Prevention of recurrent seizures may diminish the effects of hypometabolism and hypoxia; therefore, the goal is complete seizure control. Management principles for recurrent seizures associated with other conditions also apply to seizure prophylaxis in SWS. Children are initially placed on carbamazepine, with phenobarbital and phenytoin as second-line therapies, If control is not achieved, valproate or topiramate may be added to carbamazepine, with the ultimate goal of monotherapy seizure control with valproate or topiramate. Children who receive no relief from frequent, debilitating seizures are candidates for epilepsy surgery. Objective: To describe the characteristics of patients with epilepsy of Sturge Weber syndrome and its implications on the evolution in the Hospital Infantil de Mexico in the last 10 years. Method: The study design is descriptive and Transversal. We performed a review of records of patients with Sturge Weber syndrome and epilepsy in the past 10 years, evaluating the clinical characteristics of seizures and age at onset of the same, given the management and history of Pharmacotherapy . Results: We evaluated a total of 13 patients in the last 10 years. 61.5 are female and 38.5% male. The onset of the crisis with a mean age of 16.4 months, with the smallest one month and the older is 4 years. The seizure types were observed with partial onset seizures and secondarily generalized increased tone with 41.5%. 11.7% simple partial seizures, complex partial seizures 11.7%, 11.7% Tonic seizures, Infantile spasms 5.8%, atypical absence 5.8%, simple partial with motor symptoms hemicorporeal 5.8% and complex febrile seizures 5.8%. Control of epilepsy is considered when managing to have a six or more months free period of seizures. 61.5% were with proper management, and 38.4% of patients did not. The facial nerve branch V1 mostly affected was 46.1%, V2; 34.6%, V3 19.2%, 76.9% unilaterally and bilateral 23.07%. Per patient affected 38.4% of the three branches, two branches 30.7% and only one branch 30.7%. Cerebral lobe affected: Occipital 16%, Temporal 28%, Parietal 24%, Frontal 32%. The drugs used in the study group were 11 in total, in various combinations, the most used is VPA, more than a half of de cases with 54.5%, followed by the PB with 27.2%, CLB 27.2%, TPM 18.1%, OXC 18.1%, LTG 9.0%, GBP 9.0%, PHT 9.0%, CBZ 9.0%, PRM 9.0%, VGB 9.0%. Conclusion: In our study the objective was to compare the world's population with that of our institution, with respect to seizures, the age range we mentioned literature is very variable range, we observed that the age at presentation was smaller this is reflected in the outcome, showing all the patients with important developmental disorders and refractory Epilepsy. Something very important is that in several studies mentioned that the brain most affected lobe is the occipital, in our study was observed more frequently affected temporal and parietal lobe, including total hemispheric conditions, and presence of facial nevus in the three branches and some patients with bilateral involvement, this being reflected in the clinical characteristics of the crisis. The literature emphasizes that crises are more frequent Partial and West syndrome, we found a very important difference: in most widespread are the property of which some had partial onset, and only one patient manifested with epileptic encephalopathy. Most were women. A large number of our patients did not have adequate control.