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.
目的 利用磁共振彌散張量成像技術（DTI）對右側難治性顳葉癲癇（TLE）患者術前及術后腦白質各向異性分數（FA）進行縱向隨訪研究，并對其與病程等臨床癥狀之間的相關性進行分析，探討右側TLE患者的腦白質FA變化模式。 方法 2008年7月－2009年8月納入10例右側難治性TLE患者。對每個受試者采用GE 3.0 T磁共振及8通道頭線圈掃描，所有DTI圖像通過單次回波平面成像序列采集。采用基于體素分析的SPM8軟件對受試患者術前術后FA圖進行配對t檢驗，觀察難治性TLE患者腦白質變化模式。采用Pearson相關計算FA變化幅度與病程等臨床癥狀之間的相關性，經比較校正后P值＜0.05的區域為有統計學意義的區域。 結果 右側TLE患者FA值降低的區域包括左側顳下回、雙側額中回及左側殼核、右側楔葉。FA升高的區域包括左側海馬旁回、左側顳葉、右側額下回和左側中央旁小葉。相關分析發現，右側TLE患者右側額下回FA變化值與發病年齡呈負相關，左側顳下回FA變化值與術后隨訪間隔時間呈負相關。 結論 右側難治性TLE患者手術治療后大腦白質變化不僅局限于顳葉，還涉及顳葉外結構。