scholarly journals An Examination of Serum Acylcarnitine and Amino Acid Profiles at Different Time Point of Ketogenic Diet Therapy and Their Association of Ketogenic Diet Effectiveness

Nutrients ◽  
2020 ◽  
Vol 13 (1) ◽  
pp. 21
Author(s):  
Pi-Lien Hung ◽  
Ju-Li Lin ◽  
Chien Chen ◽  
Kai-Yin Hung ◽  
Tzu-Yun Hsieh ◽  
...  
Keyword(s):  
Ketogenic Diet ◽  
Retention Rate ◽  
Reduction Rate ◽  
Diet Therapy ◽  
Free Carnitine ◽  
Carnitine Level ◽  
Seizure Reduction ◽  
Amino Acid Profiles ◽  
Drug Resistant Epilepsy ◽  
Glycine Level

Background: This study aimed to identify metabolic parameters at different time points of ketogenic diet therapy (KDT) and investigate their association with response to KDT in pediatric drug-resistant epilepsy (DRE). Methods: Prospectively, twenty-nine patients (0.67~20 years old) with DRE received classic ketogenic diet with non-fasting, gradual KD initiation protocol (GRAD-KD) for 1 year were enrolled. A total of 22 patients remaining in study received blood examinations at baseline, 3rd, 6th, 9th, and 12th months of KDT. β-hydroxybutyrate, free carnitine, acylcarnitines, and amino acids were compared between responders (seizure reduction rate ≥ 50%) and non-responders (seizure reduction rate < 50%) to identify the effectiveness of KDT. Results: The 12-month retention rate was 76%. The responders after 12 months of KDT were 59% (13/22). The free carnitine level decreased significantly at 9th months (p < 0.001) but increased toward baseline without symptoms. Propionyl carnitine (C3), Isovaleryl carnitine (C5), 3-Hydroxyisovalerylcarnitine (C5:OH) and methylmalonyl carnitine (C4-DC) decreased but 3-hydroxybutyrylcarnitine (C4:OH) increased significantly at 12th months of KDT. The glycine level was persistently higher than baseline after KDT. KDT responders had lower baseline C3 and long-chain acylcarnitines, C14 and C18, as well as lower C5, C18, and leucine/isoleucine. Conclusions: KDT should be avoided in patients with non-ketotic hyperglycemia. Routine carnitine supplementation is not recommended because hypocarnitinemia was transient and asymptomatic during KDT. Better mitochondrial βoxidation function associates with greater KDT response.

Long-term effectiveness and adverse effects of ketogenic diet therapy in infants with drug-resistant epilepsy treated at a single center in Argentina

2021 ◽  
pp. 106793
Author(s):  
Marisa Armeno ◽  
Antonella Verini ◽  
Eugenia Caballero ◽  
Araceli Cresta ◽  
Gabriela Reyes Valenzuela ◽  
...  
Keyword(s):  
Adverse Effects ◽  
Ketogenic Diet ◽  
Diet Therapy ◽  
Drug Resistant ◽  
Single Center ◽  
Drug Resistant Epilepsy

Prevalence of Carnitine Deficiency and Decreased Carnitine Levels in Patients on Hemodialysis

2019 ◽  
Vol 47 (Suppl. 2) ◽  
pp. 38-44 ◽  
Author(s):  
Yoshinari Hatanaka ◽  
Terumi Higuchi ◽  
Yurie Akiya ◽  
Tomomi Horikami ◽  
Ritsukou Tei ◽  
...  
Keyword(s):  
At Risk ◽  
High Risk ◽  
Multivariate Regression ◽  
Reduction Rate ◽  
Carnitine Deficiency ◽  
Free Carnitine ◽  
Prevalence Rates ◽  
Carnitine Level ◽  
Serum Free ◽  
Free Serum

Background: Patients on hemodialysis (HD) are known to be at risk of carnitine deficiency. The aims of this study were to investigate the prevalence of carnitine deficiency in patients on dialysis and to compare the likelihood of a reduction in the serum carnitine level on HD with that on hemodiafiltration (HDF). Methods: The prevalence of carnitine deficiency, defined as a serum free carnitine level < 20 μmol/L, and that of carnitine insufficiency, defined as an acyl/free carnitine ratio > 0.4, was investigated in 150 patients on dialysis. The reduction rate of serum carnitine was then compared between HD and HDF. Results: The prevalence of carnitine deficiency and that of carnitine insufficiency was 25.3 and 86.7%, respectively. Patients at high risk of carnitine deficiency accounted for 64.7%. Multivariate regression identified an association of duration of dialysis with the free serum carnitine level. The reduction rates of serum free carnitine in HD and HDF were 64 ± 4 and 75 ± 7%, respectively (p < 0.0001). Conclusion: The prevalence rates of carnitine deficiency and carnitine insufficiency were high in patients on dialysis. The serum carnitine reduction rate was greater with HDF than with HD.

scholarly journals Ketogenic Diets for Adults with Highly Refractory Epilepsy

2017 ◽  
Vol 17 (6) ◽  
pp. 346-350 ◽  
Author(s):  
Tanya J. W. McDonald ◽  
Mackenzie C. Cervenka
Keyword(s):  
Status Epilepticus ◽  
Side Effects ◽  
Ketogenic Diet ◽  
Refractory Epilepsy ◽  
Diet Therapy ◽  
Drug Resistant ◽  
Ketogenic Diets ◽  
Current Review ◽  
Drug Resistant Epilepsy

The current review highlights the evidence supporting the use of ketogenic diets in the management of drug-resistant epilepsy and status epilepticus in adults. Ketogenic diet variants are compared and advantages and potential side effects of diet therapy are discussed.

Evaluation of the effects of ketogenic diet therapy on sleep quality in children with drug-resistant epilepsy and their mothers

2021 ◽  
Vol 124 ◽  
pp. 108327
Author(s):  
Aycan Ünalp ◽  
Bahar Toklu Baysal ◽  
Serdar Sarıtaş ◽  
Yiğithan Güzin ◽  
Selvinaz Edizer ◽  
...  
Keyword(s):  
Sleep Quality ◽  
Ketogenic Diet ◽  
Diet Therapy ◽  
Drug Resistant ◽  
Drug Resistant Epilepsy

scholarly journals Effective and safe diet therapies for Lennox-Gastaut syndrome with mitochondrial dysfunction

2020 ◽  
Vol 13 ◽  
pp. 175628641989781
Author(s):  
Ji-Hoon Na ◽  
Heung-Dong Kim ◽  
Young-Mock Lee
Keyword(s):  
Cognitive Function ◽  
Mitochondrial Dysfunction ◽  
Retention Rate ◽  
Tertiary Care ◽  
Reduction Rate ◽  
Cognitive Status ◽  
Seizure Freedom ◽  
Seizure Reduction ◽  
Gastrointestinal Problems

Background: Lennox-Gastaut syndrome (LGS) is a typical intractable form of epilepsy that most often occurs between the second and sixth year of life. This study aimed to evaluate the clinical efficacy and safety of ketogenic diet therapies (DTs) for LGS with mitochondrial dysfunction. Methods: This was a retrospective study involving 20 LGS patients with mitochondrial dysfunction who received several DTs from 2004 to 2014 at a single tertiary care center. Seizure reduction rate, cognitive function, retention rate, electroencephalography (EEG) changes, and adverse effects were examined before and after DTs. Results: The retention rates at 1 and 2 years after initiation of DTs were 45% and 40%, respectively. After 1-year follow up, we observed seizure freedom in two patients, 75% seizure reduction in two patients, 50% reduction in three patients, and 25% reduction in one patient. After 2-year follow up, the outcomes were seizure freedom in two patients, 90% seizure reduction in one patient, 75% reduction in two patients, and 50% reduction in two patients. EEG findings improved in nine patients. Nine patients were treated with DTs for 1 year; all patients demonstrated improved cognitive status. Eight patients were treated with DTs for 2 years, of whom seven had improved cognitive status. Poor tolerability of DTs was due to poor oral intake and gastrointestinal problems. Conclusions: We demonstrate that, in LGS with mitochondrial dysfunction, improvement of seizures and cognitive function are not inferior to those in other patients treated with DTs. This study showed that DTs are efficacious and feasible for LGS patients with mitochondrial dysfunction and can significantly improve their prognosis.

scholarly journals Improving the effects of ketogenic diet therapy in children with drug-resistant epilepsy

Seizure ◽  
2021 ◽  
Author(s):  
Rongrong Yang ◽  
Jialun Wen ◽  
Wenjing Wei ◽  
Haili Chen ◽  
Dezhi Cao ◽  
...  
Keyword(s):  
Ketogenic Diet ◽  
Diet Therapy ◽  
Drug Resistant ◽  
Drug Resistant Epilepsy

scholarly journals Effects of Vagus Nerve Stimulation Following Corpus Callosotomy for Patients with Drug-Resistant Epilepsy

2021 ◽  
Vol 11 (11) ◽  
pp. 1395
Author(s):  
Keisuke Hatano ◽  
Ayataka Fujimoto ◽  
Takamichi Yamamoto ◽  
Hideo Enoki ◽  
Tohru Okanishi
Keyword(s):  
Vagus Nerve ◽  
Seizure Frequency ◽  
Nerve Stimulation ◽  
Vagus Nerve Stimulation ◽  
Seizure Control ◽  
Reduction Rate ◽  
Corpus Callosotomy ◽  
Seizure Reduction ◽  
Drug Resistant Epilepsy ◽  
Univariate Analyses

Objective: The effectiveness of vagus nerve stimulation (VNS) for residual seizures after corpus callosotomy (CC) has not yet been fully investigated. We hypothesized that seizure control would be improved by VNS after CC. The purpose of this study was to compare seizure frequency between patients with implantation of a VNS generator (post-VNS group) or without VNS (non-post-VNS group) following CC. Methods: We retrospectively reviewed patients who underwent CC between January 2009 and May 2019 in our institution. We evaluated proportions of ≥50% reduction in seizure frequency (responders) and seizure reduction rate 1 and 2 years after VNS. To investigate factors related to responders, uni- and multivariate logistic regression analyses were performed regarding age, number of anti-seizure medications (ASMs), addition of novel ASMs (levetiracetam, lacosamide or perampanel), and post-VNS or non-post-VNS status. Results: Thirteen post-VNS patients and 24 non-post-VNS patients were analyzed in this study. Responder rate at 1 year after VNS differed significantly between the post-VNS group (53.9%) and non-post-VNS group (12.5%, p = 0.017). Number of ASMs at the time of CC and post-VNS were significantly associated with responders in univariate analyses (odds ratio [OR] 0.34, 95% confidence interval [CI] 0.13–0.88, p = 0.025 and OR 8.2, 95%CI 1.6–41.6, p = 0.011, respectively), whereas age, sex, seizure frequency, and addition of novel ASMs were not. In multivariate analysis, the presence of VNS procedures after CC was the only factor favorably associated with responder status (OR 82.2, 95%CI 1.55–4355.7, p = 0.03). Conclusions: VNS therapy after CC may increase the proportion of responders independent of the addition of novel ASMs.

scholarly journals Ketogenic Diet Therapy for the Treatment of Post-encephalitic and Autoimmune-Associated Epilepsies

2021 ◽  
Vol 12 ◽  
Author(s):  
Khalil S. Husari ◽  
Mackenzie C. Cervenka
Keyword(s):  
Status Epilepticus ◽  
Ketogenic Diet ◽  
Case Series ◽  
Diet Therapy ◽  
Refractory Status Epilepticus ◽  
Prior History ◽  
Seizure Freedom ◽  
Modified Atkins Diet ◽  
Feasible Option ◽  
Drug Resistant Epilepsy

Introduction: Acute Encephalitis is associated with a high risk of acute symptomatic seizures, status epilepticus, and remote symptomatic epilepsy. Ketogenic diet therapies (KDT) have been established as a feasible and safe adjunctive management of refractory- and super-refractory status epilepticus. However, the role of KDT in the chronic management of Post-encephalitic epilepsy (PE) and autoimmune-associated epilepsy (AE) is unknown. This study aims to investigate the use of KDT in patients with PE and AE.Methods: A retrospective single-center case series examining adult patients with PE and AE treated with the modified Atkins diet (MAD), a KDT commonly used by adults with drug-resistant epilepsy.Results: Ten patients with PE and AE who were treated with adjunctive MAD were included. Four patients had either confirmed or presumed viral encephalitis, five patients had seronegative AE, and one patient had GAD65 AE. The median latency between starting MAD and onset of encephalitis was 6 years (IQR: 1–10). The median duration of MAD was 10 months (IQR: 3.75–36). Three patients (30%) became seizure-free, one patient (10%) achieved 90% seizure freedom, and three patients (30%) achieved a 50–75% reduction in their baseline seizure frequency, while three patients (30%) had no significant benefit. Overall, seven patients (70%) achieved ≥50% seizure reduction.Conclusion: In addition to its established role in the treatment of RSE, KDT may be a safe and feasible option for the treatment of chronic PE and AE, particularly in those with prior history of SE. Prospective studies are warranted to explore the efficacy of KDT in management of patients with PE and AE.

Ketogenic Diet Therapy for Epilepsy Associated With Aicardi Syndrome

2021 ◽  
pp. 088307382110233
Author(s):  
Miguel A. Ramirez Sanchez ◽  
Mackenzie C. Cervenka ◽  
Stacey K. Bessone ◽  
Eric H. Kossoff
Keyword(s):  
Ketogenic Diet ◽  
Neurodevelopmental Disorder ◽  
Diet Therapy ◽  
Infantile Spasms ◽  
Johns Hopkins Hospital ◽  
Seizure Freedom ◽  
Seizure Reduction ◽  
Patients Âgés ◽  
Short Period ◽  
Aicardi Syndrome

Introduction: Aicardi syndrome is a rare neurodevelopmental disorder associated with epilepsy in females. Ketogenic diet therapy represents a possible nonpharmacologic treatment in Aicardi syndrome patients. Methods: All patients with Aicardi syndrome seen at Johns Hopkins Hospital (Baltimore, MD) and Johns Hopkins All Children’s Hospital (St Petersburg, FL) treated with ketogenic diet therapy since 1994 were evaluated retrospectively. Results: Fifteen patients, ages 4 months to 34 years, were identified. Ten (67%) patients experienced a ≥50% seizure reduction after 3 months, with 3 (20%) having a ≥90% reduction. Only 1 patient was seizure-free for a short period of time. The number of drugs tried prior to ketogenic diet therapy initiation was correlated with ≥50% seizure reduction at 3 months, 5.8 vs 2.6 in responders versus nonresponders ( P = .01). In addition, the mean number of drugs actively received also correlated, 3.0 vs 1.2, P = .005. Ketogenic diet therapy was slightly more successful in those without infantile spasms, 78% vs 50%, P = .33. Conclusion: Ketogenic diet therapy was helpful in Aicardi syndrome, although seizure freedom was rare. It was especially helpful for those who were more drug-resistant and did not have infantile spasms at ketogenic diet therapy onset.

scholarly journals Ketogenic Diet in Refractory Childhood Epilepsy

2018 ◽  
Vol 5 ◽  
pp. 2329048X1877949 ◽  
Author(s):  
Amerins Weijenberg ◽  
Margreet van Rijn ◽  
Petra M. C. Callenbach ◽  
Tom J. de Koning ◽  
Oebele F. Brouwer
Keyword(s):  
Ketogenic Diet ◽  
Retention Rate ◽  
Outpatient Setting ◽  
Secondary Outcome ◽  
Liquid Formulation ◽  
Seizure Reduction ◽  
Number Of Children ◽  
Considerable Impact ◽  
The Mean ◽  
Mean Time

Background: Ketogenic diet in children with epilepsy has a considerable impact on daily life and is usually adopted for at least 3 months. Our aim was to evaluate whether the introduction of an all-liquid ketogenic diet in an outpatient setting is feasible, and if an earlier assessment of its efficacy can be achieved. Methods: The authors conducted a prospective, observational study in a consecutive group of children with refractory epilepsy aged 2 to 14 years indicated for ketogenic diet. Ketogenic diet was started as an all-liquid formulation of the classical ketogenic diet, KetoCal 4:1 LQ, taken orally or by tube. After 6 weeks, the liquid diet was converted into solid meals. The primary outcome parameter was time-to-response (>50% seizure reduction). Secondary outcome parameters were time to achieve stable ketosis, the number of children showing a positive response, and the retention rate at 26 weeks. Results: Sixteen children were included. Four of them responded well with respect to seizure frequency, the median time-to-response was 14 days (range 7-28 days). The mean time to achieve stable ketosis was 7 days. The retention rate at 26 weeks was 50%. Of the 8 children who started this protocol orally fed, 6 completed it without requiring a nasogastric tube. Conclusions: Introduction of ketogenic diet with a liquid formulation can be accomplished in orally fed children without major complications. It allowed for fast and stable ketosis.

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