Efficacy and safety of ketogenic diet in tuberous sclerosis complex with epilepsy_Journal of Epilepsy

Authors：

 LONGWeiwei ^1,2 , JIANGLi ¹ ,  LIAOJianxiang ²

1. Department of Paediatric Neurology, Children's Hospital Affiliated to Medical University Of Chongqing, Chongqing 400014, China;
2. Department of Paediatric Neurology, Shenzhen Children's Hospital Affiliated to Medical University Of Chongqing, Shenzhen 518038, Guangdong, China;

Corresponding?author：

LIAOJianxiang, Email: epilepsycenter@vip.163.com

Keywords：

Children epilepsy; Ketogenic diet; Tuberous sclerosis complex; Efficacy

DOI：

10.7507/2096-0247.20150017

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Objective Tuberous sclerosis complex (TSC) is a multiorgan disorder and mostly associated with intractable epilepsy. Now several individual reports suggest that epilepsy in children with TSC might benefit from a ketogenic diet (KD). We prospectively studied the curative effect of 14 children with the KD in the treatment of TSC with epilepsy. Methods Between 2008 and 2015, we enrolled 14 children with TSC and epilepsy who received KD treatment in Shenzhen Children's Hospital and followed up for at least three months.Outcome was measured by the change of seizure frequency before and after the KD in the use of anticonvusant drugs, adverse effects, and change in cognitive function. Results 14 children aged 8 months to 7 years were included. 7/14 (50%) children had a > 50% reduction in seizure frequency at 3 months on the diet, 5/14 (36%) children had a seizure free response. 12/14 (86%) children with refractory epilepsy, 6/12 (50%) children had a > 50% reduction in seizure frequency, 2 children had reduced medications, one child did not use any antiepileptic drugs during KD. 6 of 12 children with developmental delays had cognitive function improvement. Conclusions KD is a generally effective and safe therapy for TSC children with epilepsy, especially for refractory epilepsy. KD could reduce antiepileptic drugs, and also improve children's cognitive function.

Citation： LONGWeiwei, JIANGLi, LIAOJianxiang. Efficacy and safety of ketogenic diet in tuberous sclerosis complex with epilepsy. Journal of Epilepsy, 2015, 1(2): 118-122. doi: 10.7507/2096-0247.20150017 Copy

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2.	廖建湘.生酮飲食療法的臨床方案.中華實用兒科臨床雜志, 2013, 28(12):881-883.
3.	王紀文, 張娜娜.生酮飲食療法的安全性.中華實用兒科臨床雜志, 2013, 28(12):886-887.
4.	Kossoff EH, Thiele EA, Pfeifer HH, et al. Tuberous sclerosis complex and the ketogenic diet. Epilepsia, 2005, 46(10):1684-1686.
5.	Roach ES, Gomez MR, Northrup H. Tuberous sclerosis complex consensus conference:revised clinical diagnostic criteria. Child Neurol, 1998, 13(7):624-628.
6.	Kossoff EH, Zupec Kania BA, Amark PE, et al. Optimal climical management of children receiving the ketogenicdiet:recommendations of the International Ketogenic Diet Study Group. Epilepsia, 2009, 50 (2):304-317.
7.	Dhamija R, Eckea S. Ketogenic diet. Can J Neurol Sci, 2013, 40(2):158-167.
8.	Suo C, Liao J, Lu X, et al. Efficacy and safety of the ketogenic diet in Chinese children. Seizure, 2013, 22(3):174-178.
9.	Coppola G, Klepper J, Ammendola E, et al. The effects of the ketogenic diet in refractory partial seizures with reference to tuberous sclerosis. Euro J Paed Neurol, 2006, 10(3):148-151.
10.	Ijff DM, Aldenkamp AP. Cognitive side-effects of antiepileptic drugs in children. Handb Clin Neurol, 2013, 111(36):707-718.
11.	Eddy CM, Rickards HE, Cavanna AE. The cognitive impact of antiepileptic drugs. The Adv Neurol Disord, 2014, 4(2):385-407.
12.	周水珍.生酮飲食治療兒童難治性癲癇.中華實用兒科臨床雜志, 2013, 28(2):883-885.
13.	朱登納, 謝蒙蒙, 王軍, 等.生酮飲食添加治療難治性癲癇36例.中國實用兒科臨床雜志, 2014, 29(19):1483-1487.
14.	Lambrechts DA, Bovens MJ, de la Parra NM, et al.Ketogenic diet effects on cognition, mood, and psychosocial adjustment in children. Acta Neurol Scand, 2013, 127(2):103-108.
15.	Gha-hyun Lee, Bo-mi Kim, Joong Koo Kang, et al. Loss of the initial efficacy of levetiracetam in patients with refractory epilepsy. Seizure, 2013, 22(3):185-188.
16.	Nabil J, AzarAndre H Lagrange, Lily Wang, et al. Transient improvement after brief antiepileptic drug withdrawal in the epilepsy monitoring unit-possible ralationship to AED tolerance. Epilepsia, 2010, 51(5):811-817.

1. Curatolo P, Bombardieri R, Jozwiak S. Tuberous sclerosis. Lancet, 2008, 372(128):657-668.
2. 廖建湘.生酮飲食療法的臨床方案.中華實用兒科臨床雜志, 2013, 28(12):881-883.
3. 王紀文, 張娜娜.生酮飲食療法的安全性.中華實用兒科臨床雜志, 2013, 28(12):886-887.
4. Kossoff EH, Thiele EA, Pfeifer HH, et al. Tuberous sclerosis complex and the ketogenic diet. Epilepsia, 2005, 46(10):1684-1686.
5. Roach ES, Gomez MR, Northrup H. Tuberous sclerosis complex consensus conference:revised clinical diagnostic criteria. Child Neurol, 1998, 13(7):624-628.
6. Kossoff EH, Zupec Kania BA, Amark PE, et al. Optimal climical management of children receiving the ketogenicdiet:recommendations of the International Ketogenic Diet Study Group. Epilepsia, 2009, 50 (2):304-317.
7. Dhamija R, Eckea S. Ketogenic diet. Can J Neurol Sci, 2013, 40(2):158-167.
8. Suo C, Liao J, Lu X, et al. Efficacy and safety of the ketogenic diet in Chinese children. Seizure, 2013, 22(3):174-178.
9. Coppola G, Klepper J, Ammendola E, et al. The effects of the ketogenic diet in refractory partial seizures with reference to tuberous sclerosis. Euro J Paed Neurol, 2006, 10(3):148-151.
10. Ijff DM, Aldenkamp AP. Cognitive side-effects of antiepileptic drugs in children. Handb Clin Neurol, 2013, 111(36):707-718.
11. Eddy CM, Rickards HE, Cavanna AE. The cognitive impact of antiepileptic drugs. The Adv Neurol Disord, 2014, 4(2):385-407.
12. 周水珍.生酮飲食治療兒童難治性癲癇.中華實用兒科臨床雜志, 2013, 28(2):883-885.
13. 朱登納, 謝蒙蒙, 王軍, 等.生酮飲食添加治療難治性癲癇36例.中國實用兒科臨床雜志, 2014, 29(19):1483-1487.
14. Lambrechts DA, Bovens MJ, de la Parra NM, et al.Ketogenic diet effects on cognition, mood, and psychosocial adjustment in children. Acta Neurol Scand, 2013, 127(2):103-108.
15. Gha-hyun Lee, Bo-mi Kim, Joong Koo Kang, et al. Loss of the initial efficacy of levetiracetam in patients with refractory epilepsy. Seizure, 2013, 22(3):185-188.
16. Nabil J, AzarAndre H Lagrange, Lily Wang, et al. Transient improvement after brief antiepileptic drug withdrawal in the epilepsy monitoring unit-possible ralationship to AED tolerance. Epilepsia, 2010, 51(5):811-817.

Journal of Epilepsy

Efficacy and safety of ketogenic diet in tuberous sclerosis complex with epilepsy

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