Objective To investigate the clinical features and long-term prognosis of children with symptomatic epilepsy complicated by encephalofacial angiomatosis. Methods A total of 38 children with Sturge-Weber syndrome (SWS) complicated by symptomatic epilepsy who were diagnosed and treated in the Children's Hospital of Soochow University from January 2011 to June 2020 were selected as the research objects. Their clinical data were collected, and long-term follow-up studies were carried out. Results The clinical manifestations of SWS complicated by symptomatic epilepsy were diverse, with focal seizures being the most common (86.84%). Abnormalities were found in the cranial CT and MRI of all 38 children, mainly involving the parietal lobe and occipital lobe. Among the 38 children undergoing electroencephalogram (EEG) examinations, 31 had abnormal results. Among the abnormal EEGs, the background waves were asymmetrical on the left and right sides in 28 cases (the amplitude on the affected side was lower), 8 cases showed unilateral discharges to varying degrees, and 1 case showed bilateral high-amplitude discharges. Among the 38 cases, 2 did not use antiepileptic drugs, and 36 were treated with antiepileptic drugs, mainly with oxcarbazepine, topiramate, and levetiracetam. Eventually, 2 children were given surgical treatment due to drug-resistant epilepsy. Among the 38 cases, 19 (50.00%) were diagnosed with refractory epilepsy. Epilepsy occurring before the age of 2 are prone to develop into refractory epilepsy (P<0.05). All 38 children had sequelae to varying degrees, including 25 cases (65.79%) with severe sequelae and 13 cases (34.21%) with milder sequelae. Moreover, the earlier the age of epilepsy onset in children, the more likely it was to leave severe sequelae in the later stage (P=0.001). Conclusion Children with Sturge-Weber syndrome complicated by symptomatic epilepsy mainly have focal seizures. About half of them suffer from refractory epilepsy. The earlier the age of epileptic seizures, the more likely it is to develop into refractory epilepsy. The long-term prognosis of such children is rather poor. Among the 38 cases followed up, all had sequelae of varying degrees.
ObjectiveTo observe the clinical efficacy and safety of Lacosamide (LCM) monotherapy in treating Benign epilepsy with centro-temporal spikes (BECT) at children. MethodsThe present research included 57 children with BECT who were treated with LCM monotherapy in the Children's Hospital Affiliated to Soochow University from June 2020 to June 2021. Among them, 26 were males and 31 were females, with an average age of (7.5±2.3) years. Analyze the efficacy and adverse reactions of 1, 2, 4, 6, 9 and 12 months after LCM treatment. The starting dose of LCM was 2 mg/(kg·d); increased 2 mg/(kg·d) every seven days; and titrated up to 4 ~ 8 mg/(kg·d) in children with weight ≥30 kg and <50 kg; titrated up to 6 ~ 12 mg/(kg·d) in children with weight ≥11 kg and <30 kg.ResultThe total effective rate was 94.12%; the cumulative control rate after 12-month medication was 86.27%; and the retention rate was 89.47%. Those all showed a higher rate. ConclusionLCM is significantly effective to BECT with less adverse reactions and more safety. It has high clinical application value.
ObjectiveTo analyze and evaluate the clinical characteristics, efficacy, and tolerability of lacosamide monotherapy in the treatment of primary paroxysmal kinesigenic dyskinesia (PKD). Methods The Clinical data of children with primary PKD who received lacosamide monotherapy between July 2021 and June 2025 in the Children's Hospital of Soochow University were analyzed, and their efficacy and tolerability were followed up. Results During the study period, a total of 7 children with primary PKD received lacosamide monotherapy. Among them, 4 had simple-type PKD and 3 had complex-type PKD; 2 had familial PKD and 5 had sporadic PKD; 4 were male and 3 were female. The age of onset was 11.0 (0.5 ~ 12.0) years. All 7 cases presented with dystonia as the form of onset, with 1 case also exhibiting athetosis. The attack frequency ranged from once every few days or weeks to 20 times per day, with a duration of 3 ~ 60 seconds. Bilateral limb involvement was most common, and 2 cases also had facial involvement. In auxiliary examinations, 3 cases showed abnormal EEG results, and 4 cases had abnormal genetic test results. Among genetic test results, 2 cases had PRRT2 gene mutations, 1 had a TMEM151A gene mutation, and 1 had a CLCN1 gene mutation. During lacosamide treatment, the initial and long-term maintenance doses were similar, at 2.87 (0.50 ~ 4.44) mg/(kg·d) and 2.67 (0.50 ~ 4.00) mg/(kg·d), respectively. At both the 4-week and long-term follow-ups, all 7 children showed good efficacy and tolerability.ConclusionLacosamide is effective and well-tolerated in children with both familial and sporadic, simple-type and complex-type primary PKD. A low dose of 2.67 (0.50 ~ 4.00) mg/(kg·d) is sufficient to control attacks, which is lower than the minimum maintenance dose of 4 ~ 6 mg/(kg·d) used in the treatment of epilepsy with lacosamide.