结节性硬化症中耐药性癫痫的预测因素
Predictors of Drug-Resistant Epilepsy in Tuberous Sclerosis Complex.
作者信息
Jeong Anna, Nakagawa Jo Anne, Wong Michael
机构信息
1 Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA.
2 Tuberous Sclerosis Alliance, Silver Spring, MD, USA.
出版信息
J Child Neurol. 2017 Dec;32(14):1092-1098. doi: 10.1177/0883073817737446.
Utilizing the multicenter TSC (tuberous sclerosis complex) Natural History Database including 2034 subjects, this study aimed to identify predictors of drug-resistant epilepsy in TSC. Basic epilepsy data were available for 1965 individuals in the database. Supplemental data were further collected from 1546 of these subjects through directed site queries, addressing additional epilepsy characteristics including the presence of drug-resistant epilepsy, therapies trialed, and outcomes of specific therapies. Epilepsy was reported in 86.4% of individuals with TSC. Infantile spasms were reported in 45.2% of individuals and focal seizures were reported in 84.4% of individuals. In those with focal epilepsy, drug resistance was reported in 59.6%, with focal seizure onset prior to age 1 year (odds ratio [OR] 1.9, confidence interval [CI] 1.4-2.5, P < .001), infantile spasms (OR 2.0, CI 1.5-2.5, P < 0.001), and infantile spasms incompletely responsive to therapy (OR 47.6, CI 6.7-333.3, P < 0.001) being associated with an increased likelihood of drug resistance.
本研究利用包含2034名受试者的多中心结节性硬化症（TSC）自然史数据库，旨在确定TSC中耐药性癫痫的预测因素。数据库中有1965名个体的基本癫痫数据。通过定向站点查询，从其中1546名受试者中进一步收集补充数据，涉及额外的癫痫特征，包括耐药性癫痫的存在、试验的治疗方法以及特定治疗的结果。86.4%的TSC个体报告有癫痫。45.2%的个体报告有婴儿痉挛症，84.4%的个体报告有局灶性癫痫发作。在局灶性癫痫患者中，59.6%报告有耐药性，1岁前出现局灶性癫痫发作（比值比[OR]1.9，置信区间[CI]1.4 - 2.5，P <.001）、婴儿痉挛症（OR 2.0，CI 1.5 - 2.5，P < 0.001）以及对治疗反应不完全的婴儿痉挛症（OR 47.6，CI 6.7 - 333.3，P < 0.001）与耐药性可能性增加相关。
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