Hypocarnitinemia and its effect on seizure control in adult patients with intractable epilepsy on the modified Atkins diet.
Hypocarnitinemia and its effect on seizure control in adult patients with intractable epilepsy on the modified Atkins diet.
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DOI：
10.3389/fnut.2023.1304209
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发表时间：
2023
影响因子：
5
通讯作者：
Felton, Elizabeth A.
中科院分区：
文献类型：
--
作者：
Chu, Daniel Y.;Ravelli, Michele N.;Faltersack, Kelly M.;Woods, Arron L.;Almane, Dace;Li, Zhanhai;Sampene, Emmanuel;Felton, Elizabeth A.
先前的研究已经证明了改良阿特金斯饮食（MAD）在减弱难治性癫痫患者癫痫发作方面的安全性和有效性。MAD通过实现酮症起作用，酮症严重依赖于代谢化合物肉毒碱，以促进长链脂肪酸穿过线粒体进行β-氧化。肉毒碱对生酮饮食疗法的影响在目前的文献中还没有很好的定义。因此，我们的研究目的是探讨低肉毒碱血症对MAD疗效的影响。进行了回顾性病历审查，并对58名接受MAD治疗的成人癫痫患者进行了评价。采用广义线性混合效应模型比较低肉毒碱状态组与正常肉毒碱组在基线、3-9个月随访（时间点1）、1-2年随访（时间点2）和2+年随访（时间点3）期间的患者体重指数、癫痫发作频率和严重程度、抗癫痫发作药物数量、β-羟基丁酸酯、甘油三酯和肉毒碱水平。我们的研究显示，38.3%的成人癫痫患者MAD后经历了低游离肉毒碱在某些时候通过饮食治疗的过程。在时间点2低肉毒碱血症的患者表现出显着的百分比癫痫发作增加，而癫痫发作继续减少在正常肉毒碱组。与正常肉毒碱组相比，低肉毒碱组在时间点1的空腹甘油三酯水平显著增加。低肉毒碱组和正常肉毒碱组的BHB、BMI、癫痫发作严重程度和ASM数量的变化无显著差异。这可能是重要的临床医生监测低肉毒碱血症在成人MAD和低时提供肉毒碱补充。对肉毒碱和MAD的进一步调查可能会通知补充肉毒碱的临床决策，以最大限度地提高MAD治疗的疗效。
Previous studies have demonstrated the safety and efficacy of the modified Atkins diet (MAD) in attenuating seizures in patients with intractable epilepsy. MAD works by achieving ketosis, which is heavily dependent on the metabolic compound, carnitine, to facilitate the transport of long-chain fatty acids across the mitochondria for beta-oxidation. The effect of carnitine on ketogenic diet therapy is not well-defined in the current literature. Thus, the purpose of our study is to investigate the effects of hypocarnitinemia on the efficacy of MAD. A retrospective chart review was conducted, and 58 adults with epilepsy undergoing MAD were evaluated. Generalized linear mixed effects models were used to compare the low carnitine status with normal carnitine group in patient measures of body mass index, seizure frequency and severity, number of anti-seizure medications, beta-hydroxybutyrate, triglyceride, and carnitine levels across baseline, 3–9-month follow-up (timepoint 1), 1-2-year follow-up (timepoint 2), and 2+ year follow-up (timepoint 3). Our study revealed that 38.3% of adult patients with epilepsy following MAD experienced low free carnitine at some point through the course of diet therapy. Patients with hypocarnitinemia at timepoint 2 showed a significant percent seizure increase while seizures continued to decrease in the normal carnitine group. Fasting triglyceride levels at timepoint 1 were significantly increased in the low carnitine group compared to normal carnitine group. Change in BHB, BMI, seizure severity, and number of ASMs showcased no significant differences between the low and normal carnitine groups. It may be important for clinicians to monitor for hypocarnitinemia in adults on MAD and provide carnitine supplementation when low. Further investigations into carnitine and MAD may inform clinical decisions on carnitine supplementation to maximize the efficacy of MAD therapy.
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影响因子：
2.6
作者：
Roehl, Kelly;Falco-Walter, Jessica;Balabanov, Antoaneta
通讯作者：
Balabanov, Antoaneta
影响因子：
3
作者：
Kossoff EH;Zupec-Kania BA;Auvin S;Ballaban-Gil KR;Christina Bergqvist AG;Blackford R;Buchhalter JR;Caraballo RH;Cross JH;Dahlin MG;Donner EJ;Guzel O;Jehle RS;Klepper J;Kang HC;Lambrechts DA;Liu YMC;Nathan JK;Nordli DR Jr;Pfeifer HH;Rho JM;Scheffer IE;Sharma S;Stafstrom CE;Thiele EA;Turner Z;Vaccarezza MM;van der Louw EJTM;Veggiotti P;Wheless JW;Wirrell EC;Charlie Foundation;Matthew's Friends;Practice Committee of the Child Neurology Society
通讯作者：
Practice Committee of the Child Neurology Society
影响因子：
4.6
作者：
Durazzo, Alessandra;Lucarini, Massimo;Santini, Antonello
通讯作者：
Santini, Antonello
影响因子：
1.9
作者：
Chen, Wendy;Kossoff, Eric H.
通讯作者：
Kossoff, Eric H.
影响因子：
5.6
作者：
Kverneland, Magnhild;Molteberg, Ellen;Nakken, Karl O.
通讯作者：
Nakken, Karl O.