Tuberous sclerosis complex and the ketogenic diet
Tuberous sclerosis complex and the ketogenic diet
复制标题
DOI：
10.1111/j.1528-1167.2005.00266.x
复制
发表时间：
2005-10-01
期刊：
影响因子：
5.6
通讯作者：
Freeman, JM
中科院分区：
文献类型：
--
作者：
Kossoff, EH;Thiele, EA;Freeman, JM
目的：多发性硬化症（TSC）是一种经常与难治性早发性癫痫相关的疾病，通常首先被视为婴儿痉挛症。如果药物治疗失败，没有明确的致痫结节被确定，非药物治疗往往是尝试。使用生酮饮食专门为儿童TSC和癫痫尚未previously described.Methods：一个图表审查与生酮饮食治疗的TSC患者在约翰霍普金斯医院和马萨诸塞州总hospital.Results：12名儿童，年龄8个月至18岁，进行了鉴定。11名（92%）儿童在6个月的饮食中癫痫发作减少了50%以上，8名（67%）有90%以上的反应。5名儿童至少有5个月的无症状反应。饮食持续时间范围从2个月到5年（平均，2年）。结论：在这个有限的持续时间的病例系列的12例，生酮饮食是一种普遍有效的治疗方式，偶尔出现在TSC儿童的顽固性癫痫。
Purpose: Tuberous sclerosis complex (TSC) is a condition that is frequently associated with intractable, early-onset epilepsy, and often is first seen as infantile spasms. If medications fail and no clear epileptogenic tuber is identified, nonpharmacologic therapies are often attempted. The use of the ketogenic diet specifically for children with TSC and epilepsy has not been previously described.Methods: A chart review was performed of patients with TSC treated with the ketogenic diet over a 5-year period at Johns Hopkins Hospital and Massachusetts General Hospital.Results: Twelve children, ages 8 months to 18 years, were identified. Eleven (92%) children had a > 50% reduction in their seizures at 6 months on the diet, and 8 (67%) had a > 90% response. Five children had at least a 5-month seizure-free response. Diet duration ranged from 2 months to 5 years (mean, 2 years).Conclusions: In this limited-duration case series of 12 patients, the ketogenic diet was a generally effective therapeutic modality for the intractable epilepsy occasionally seen in children with TSC.