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The ketogenic diet (KD) can have a negative impact on the linear growth and body composition of children. The aims of this study were to review two centers' experience with children who developed height deceleration on the KD and determine if the height deceleration was secondary to growth hormone deficiency (GHD), and if growth hormone therapy (GHT) would be effective and safe (not altering ketosis or seizure frequency). Retrospective chart reviews were performed on patients with KD referred to Endocrinology between 2013 and 2018. Seventeen children were identified. Data reviewed included: demographics, growth velocity, KD ratio, protein/calorie intake, lab results, GH dosage, Tanner stage, and seizure frequency, and endocrine recommendations. Descriptive statistics were performed. Of the 17 children referred to the Endocrine Division, seven children were growth hormone deficient and began GHT. Data were provided for six patients (2 males, 4 females; age 2-7 years at the start of KD) on the KD for >6 years and on GHT for >4 years. Growth for all patients stabilized or increased. IGF-1 z-scores normalized. GHT did not affect seizure frequency or ketosis. GHT in those with GHD can be an appropriate option allowing better growth while still maintaining ketogenic therapy and seizure control. PLAIN LANGUAGE SUMMARY: The KD can be an effective treatment for difficult-to-control epilepsy and some disorders of carbohydrate metabolism. The KD can adversely affect the linear growth (height) of children. This case series reviewed six patients who had slow linear growth. It was found that all six children had growth hormone deficiency, grew better with growth hormone treatments, and that their seizures and ketone levels were not affected.
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Dieta Cetogênica , Hormônio do Crescimento Humano , Humanos , Feminino , Masculino , Criança , Hormônio do Crescimento Humano/deficiência , Hormônio do Crescimento Humano/administração & dosagem , Hormônio do Crescimento Humano/uso terapêutico , Pré-Escolar , Estudos Retrospectivos , Transtornos do Crescimento/dietoterapia , Fator de Crescimento Insulin-Like I/análise , Fator de Crescimento Insulin-Like I/metabolismo , Fator de Crescimento Insulin-Like I/deficiência , Estatura , Epilepsia/dietoterapiaRESUMO
BACKGROUND: Ketogenic diets (KDs) are safe and tolerable in people with multiple sclerosis (MS). While many patient-reported and clinical benefits are noted, the sustainability of these diets outside of a clinical trial is unknown. AIMS: Evaluate patient perceptions of the KD following intervention, determine the degree of adherence to KDs post-trial, and examine what factors increase the likelihood of KD continuation following the structured diet intervention trial. METHODS: Sixty-five subjects with relapsing MS previously enrolled into a 6-month prospective, intention-to-treat KD intervention. Following the 6-month trial, subjects were asked to return for a 3-month post-study follow-up, at which time patient reported outcomes, dietary recall, clinical outcome measures, and laboratory values were repeated. In addition, subjects completed a survey to evaluate sustained and attenuated benefits following completion of the intervention phase of the trial. RESULTS: Fifty-two subjects (81%) returned for the 3-month post-KD intervention visit. Twenty-one percent reported continued adherence to a strict KD and an additional 37% reported adhering to a liberalized, less restrictive form of the KD. Those subjects with greater reductions in body mass index (BMI) and fatigue at 6-months on-diet were more likely to continue on KD following trial completion. Using intention-to-treat analysis, patient-reported and clinical outcomes at 3-months post-trial remained significantly improved from baseline (pre-KD), though the degree of improvement was slightly attenuated relative to outcomes at 6-months on KD. Regardless of diet type following the KD intervention, dietary patterns shifted toward greater protein and polyunsaturated fats and less carbohydrate/added sugar consumption. CONCLUSIONS: Following the 6-month KD intervention study, the majority of subjects elected to continue on KD, though many pursued a more liberal limit for carbohydrate restriction. Those who experienced a greater reduction in BMI or fatigue were more likely to continue with strict KD. The 6-month KD intervention induced persistent changes to dietary habits in the months following study completion. TRIAL REGISTRATION INFORMATION: Registered on Clinicaltrials.gov under registration number NCT03718247, posted on Oct 24, 2018. First patient enrollment date: Nov 1, 2018. Link: https://clinicaltrials.gov/ct2/show/NCT03718247?term=NCT03718247&draw=2&rank=1.
Assuntos
Dieta Cetogênica , Esclerose Múltipla , Humanos , Estudos Prospectivos , Carboidratos , FadigaRESUMO
BACKGROUND: Dietary changes impact human physiology and immune function and have potential as therapeutic strategies. OBJECTIVE: Assess the tolerability of a ketogenic diet (KD) in patients with relapsing multiple sclerosis (MS) and define the impact on laboratory and clinical outcome metrics. METHODS: Sixty-five subjects with relapsing MS enrolled into a 6-month prospective, intention-to-treat KD intervention. Adherence was monitored with daily urine ketone testing. At baseline, fatigue, depression and quality of life (QoL) scores were obtained in addition to fasting adipokines and MS-related clinical outcome metrics. Baseline metrics were repeated at 3 and/or 6 months on-diet. RESULTS: Eighty-three percent of participants adhered to the KD for the study duration. Subjects exhibited significant reductions in fat mass and showed a nearly 50% decline in self-reported fatigue and depression scores. MS QoL physical health (67±16 vs 79±12, p<0.001) and mental health (71±17 vs 82±11, p<0.001) composite scores increased on-diet. Significant improvements were noted in Expanded Disability Status Scale scores (2.3±0.9 vs 1.9±1.1, p<0.001), 6-minute walk (1631±302 vs 1733±330 ft, p<0.001) and Nine-Hole Peg Test (21.5±3.6 vs 20.3±3.7 s, p<0.001). Serum leptin was lower (25.5±15.7 vs 14.0±11.7 ng/mL, p<0.001) and adiponectin was higher (11.4±7.8 vs 13.5±8.4 µg/mL, p=0.002) on the KD. CONCLUSION: KDs are safe and tolerable over a 6-month study period and yield improvements in body composition, fatigue, depression, QoL, neurological disability and adipose-related inflammation in persons living with relapsing MS. TRIAL REGISTRATION INFORMATION: Registered on ClinicalTrials.gov under registration number NCT03718247, posted on 24 October 2018. First patient enrolment date: 1 November 2018. Link: https://clinicaltrials.gov/ct2/show/NCT03718247?term=NCT03718247&draw=2&rank=1.
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Dieta Cetogênica , Esclerose Múltipla Recidivante-Remitente , Esclerose Múltipla , Dieta Cetogênica/efeitos adversos , Fadiga , Humanos , Esclerose Múltipla Recidivante-Remitente/psicologia , Estudos Prospectivos , Qualidade de VidaRESUMO
PURPOSE: The ketogenic diet (KD) is initiated emergently in the intensive care unit (ICU) for patients with super refractory status epilepticus (SRSE) and epileptic encephalopathies (EE). However, few data are available regarding safety, effectiveness, and long-term outcomes. METHODS: We performed a retrospective cohort study of consecutive patients with KD initiated in the ICU from 2010 to 2018 for SRSE and EE. We characterized time to ketosis, adverse effects, and seizure outcomes. Responders were defined as having ≥50 % reduction in seizure frequency compared to prior to KD initiation. RESULTS: We identified 29 patients. KD was initiated for SRSE in 12 patients, EE in 8 patients, and EE with SRSE in 9 patients. KD was initiated after a median of 9 days. Ketosis was achieved 2 days faster in fasted patients (p < 0.0001). All patients had at least 1 KD-related adverse effect, most often hypoglycemia, constipation, or acidosis. There was ≥50 % reduction in seizure frequency compared to prior to KD initiation by 1 week in 17/28 patients, seizure-freedom by 2 weeks in 7/28 patients, and weaned off anesthetics in 11/17 patients. All KD-responders at 1 month had continued response at 6 months. Mortality at 1 year was 24 %. There was no difference in KD response or mortality between KD indication groups. CONCLUSION: Emergent KD initiation in the ICU is feasible, safe, and often effective for SRSE and EE. Expected adverse effects were common but treatable. Morbidity and mortality in this group was high. A ≥ 50 % reduction in seizure is achieved in most responders by 1-2 weeks.
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Dieta Cetogênica , Estado Epiléptico , Criança , Humanos , Unidades de Terapia Intensiva , Estudos Retrospectivos , Convulsões , Resultado do TratamentoRESUMO
Treatment-resistant epilepsy affects 30% of children with epilepsy and results in significantly reduced quality of life. The ketogenic diets offer a chance for significant seizure reduction and seizure freedom. Compliance is strongly linked to the effectiveness of these treatments. The high-fat and low-carbohydrate content of the ketogenic diets makes creating and cooking palatable meals challenging. Keto centers typically support caretakers with recipes, but do not have a kitchen to provide hands-on education. Hence, our program built a ketogenic kitchen in 2013. The purpose of this study was to assess the effects of the kitchen on the quality of our education and confidence of caretakers during both initiation and ongoing outpatient support of the ketogenic diets. An anonymous survey of 37 questions was created using Survey Monkey, with a 5-point scale or yes-no responses. Families whose children have been a part of our dietary treatment program from 2014 to 2016, reachable by e-mail, were asked to take the survey. The data were analyzed using descriptive statistics. Seventy-seven families completed our survey. The overall quality of the classes taught by the dietitians improved with the use of the Ketogenic Teaching Kitchen. Hands-on cooking classes enhanced the learning experience, making our new ketogenic diet families noticeably more confident preparing meals at the time of discharge. The Keto Teaching Kitchen has greatly enhanced our dietary treatment program. We believe that all keto centers would benefit from access to a teaching kitchen.
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Dieta Cetogênica/métodos , Epilepsia Resistente a Medicamentos/dietoterapia , Pais/educação , Qualidade de Vida , Criança , Feminino , Humanos , MasculinoRESUMO
Objective: To assess the safety and tolerability of a modified Atkins diet (KDMAD), a type of ketogenic diet (KD), in subjects with relapsing MS while exploring potential benefits of KDs in MS. Methods: Twenty subjects with relapsing MS enrolled into a 6-month, single-arm, open-label study of the KDMAD. Adherence to KDMAD was objectively monitored by daily urine ketone testing. Fatigue and depression scores and fasting adipokines were obtained at baseline and on diet. Brain MRI was obtained at baseline and 6 months. Intention to treat was used for primary data analysis, and a per-protocol approach was used for secondary analysis. Results: No subject experienced worsening disease on diet. Nineteen subjects (95%) adhered to KDMAD for 3 months and 15 (75%) adhered for 6 months. Anthropometric improvements were noted on KDMAD, with reductions in body mass index and total fat mass (p < 0.0001). Fatigue (p = 0.002) and depression scores (p = 0.003) were improved. Serologic leptin was significantly lower at 3 months (p < 0.0001) on diet. Conclusions: KDMAD is safe, feasible to study, and well tolerated in subjects with relapsing MS. KDMAD improves fatigue and depression while also promoting weight loss and reducing serologic proinflammatory adipokines. Classification of evidence: The study is rated Class IV because of the absence of a non-KD control group.
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Dieta Rica em Proteínas e Pobre em Carboidratos/efeitos adversos , Dieta Cetogênica/efeitos adversos , Esclerose Múltipla Recidivante-Remitente/dietoterapia , Adipocinas , Adolescente , Adulto , Antropometria , Índice de Massa Corporal , Depressão/prevenção & controle , Fadiga/prevenção & controle , Feminino , Humanos , Inflamação/prevenção & controle , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla Recidivante-Remitente/sangue , Projetos Piloto , Resultado do Tratamento , Redução de PesoRESUMO
OBJECTIVE: Few studies have examined the long-term sustainability of complete seizure freedom on the ketogenic diet (KD). The purpose of this study was to describe the risk of seizure recurrence in children who achieved at least 1 month of seizure freedom on the KD, and to assess clinical features associated with sustained seizure freedom. METHODS: Records of patients initiated on the KD at The Children's Hospital of Philadelphia (CHOP) from 1991 to 2009 were reviewed. Subjects who attained seizure freedom for at least 1 month within 2 years were included in the study. Seizure frequency was recorded based on caregiver-reported seizure diaries as unchanged, improved, or worse compared to baseline. Those patients with seizure freedom ≥1 year were compared to those with seizure freedom <1 year in terms of demographics, age of seizure onset, number of antiepileptic drugs (AEDs) prior to KD, and epilepsy classification. RESULTS: Of 276 patients initiated on the KD, 65 patients (24%) attained seizure freedom for a minimum of 1 month. The majority of these patients had daily seizures. The median time to seizure freedom after KD initiation was 1.5 months. Seizures recurred in 53 patients (82%), with a median time to seizure recurrence of 3 months. However, seizure frequency after initial recurrence remained far less than baseline. No clinical features were identified as risk factors for seizure recurrence. SIGNIFICANCE: Seizure recurrence on the KD after 1 month of seizure freedom most often occurred as occasional breakthrough seizures and not a return to baseline seizure frequency. This study provides evidence to support the continued use of the KD in patients with initial seizure freedom even after breakthrough seizures. A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.
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Dieta Cetogênica/métodos , Convulsões/dietoterapia , Convulsões/diagnóstico , Criança , Pré-Escolar , Estudos de Coortes , Dieta Cetogênica/tendências , Feminino , Seguimentos , Humanos , Masculino , Estudos Retrospectivos , Fatores de Risco , Prevenção Secundária , Convulsões/fisiopatologiaRESUMO
BACKGROUND: The Ketogenic Diet (KD) is an effective, alternative treatment for refractory epilepsy. This high fat, low protein and carbohydrate diet mimics the metabolic and hormonal changes that are associated with fasting. AIMS: To maximize the effectiveness of the KD, each meal is precisely planned, calculated, and weighed to within 0.1 gram for the average three-year duration of treatment. Managing the KD is time-consuming and may deter caretakers and patients from pursuing or continuing this treatment. Thus, we investigated methods of planning KD faster and making the process more portable through mobile applications. METHODS: Nutritional data was gathered from the United States Department of Agriculture (USDA) Nutrient Database. User selected foods are converted into linear equations with n variables and three constraints: prescribed fat content, prescribed protein content, and prescribed carbohydrate content. Techniques are applied to derive the solutions to the underdetermined system depending on the number of foods chosen. RESULTS: The method was implemented on an iOS device and tested with varieties of foods and different number of foods selected. With each case, the application's constructed meal plan was within 95% precision of the KD requirements. CONCLUSION: In this study, we attempt to reduce the time needed to calculate a meal by automating the computation of the KD via a linear algebra model. We improve upon previous KD calculators by offering optimal suggestions and incorporating the USDA database. We believe this mobile application will help make the KD and other dietary treatment preparations less time consuming and more convenient.
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Folate and vitamin B(12) metabolism are essential for de novo purine synthesis, and several defects in these pathways have been associated with immunodeficiency. Here we describe the occurrence of severe combined immunodeficiency (SCID) with megaloblastic anemia, leukopenia, atypical hemolytic uremic syndrome, and neurologic abnormalities in which hydroxocobalamin and folate therapy provided partial immune reconstitution. Whole exome sequencing identified compound heterozygous mutations in the MTHFD1 gene, which encodes a trifunctional protein essential for processing of single-carbon folate derivatives. We now report the immunologic details of this novel genetic cause of SCID and the response to targeted metabolic supplementation therapies. This finding expands the known metabolic causes of SCID and presents an important diagnostic consideration given the positive impact of therapy.
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Análise Mutacional de DNA , Metilenotetra-Hidrofolato Desidrogenase (NADP)/genética , Imunodeficiência Combinada Severa/genética , 3-Hidroxiacil-CoA Desidrogenases/deficiência , 3-Hidroxiacil-CoA Desidrogenases/genética , Anemia Megaloblástica/diagnóstico , Anemia Megaloblástica/tratamento farmacológico , Anemia Megaloblástica/genética , Exame de Medula Óssea , Cardiomiopatias/diagnóstico , Cardiomiopatias/tratamento farmacológico , Cardiomiopatias/genética , Terapia Combinada , Combinação de Medicamentos , Quimioterapia Combinada , Exoma/genética , Feminino , Triagem de Portadores Genéticos , Humanos , Hidroxocobalamina/uso terapêutico , Imunização Passiva , Lactente , Recém-Nascido , Leucopenia/diagnóstico , Leucopenia/tratamento farmacológico , Leucopenia/genética , Erros Inatos do Metabolismo Lipídico/diagnóstico , Erros Inatos do Metabolismo Lipídico/tratamento farmacológico , Erros Inatos do Metabolismo Lipídico/genética , Antígenos de Histocompatibilidade Menor , Miopatias Mitocondriais , Proteína Mitocondrial Trifuncional/deficiência , Doenças do Sistema Nervoso , Infecções Oportunistas/diagnóstico , Infecções Oportunistas/tratamento farmacológico , Infecções Oportunistas/genética , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/tratamento farmacológico , Doenças do Sistema Nervoso Periférico/genética , Pneumonia por Pneumocystis/diagnóstico , Pneumonia por Pneumocystis/tratamento farmacológico , Pneumonia por Pneumocystis/genética , Retinose Pigmentar/diagnóstico , Retinose Pigmentar/tratamento farmacológico , Retinose Pigmentar/genética , Rabdomiólise , Análise de Sequência de DNA , Imunodeficiência Combinada Severa/diagnóstico , Imunodeficiência Combinada Severa/tratamento farmacológico , Sulfadoxina/uso terapêutico , Trimetoprima/uso terapêutico , Vitamina B 12/uso terapêuticoRESUMO
Myoclonic astatic epilepsy (MAE) is a rare childhood generalized epilepsy syndrome of unknown incidence and etiology. Onset may be explosive with a myriad of different seizure types and children may become severely affected with an epileptic encephalopathy. This disorder may be particularly sensitive to the ketogenic diet (KD). This article will briefly review the background, diagnostic criteria's and our current information regarding the use of dietary therapies in MAE.
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Dieta Cetogênica , Epilepsias Mioclônicas/dietoterapia , Idade de Início , Animais , Epilepsias Mioclônicas/diagnóstico , Epilepsia Generalizada/diagnóstico , Epilepsia Generalizada/dietoterapia , Humanos , Convulsões/dietoterapiaRESUMO
The ketogenic diet (KD) is an effective treatment for epilepsy and like other treatments it is not without side effects. The side effects encountered are related to the diet composition and the radical metabolic changes that results from a high fat, low carbohydrate and protein diet. Short-term side effects are well documented. Long-term side effects are not as well documented but since the last "international symposium on dietary therapies for epilepsy and other neurological disorders", there are now more prospective and longitudinal data. Monitoring practices and treatments will be discussed and compared to the International Ketogenic Diet Consensus Statement (IKDCS) from 2008.
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Dieta Cetogênica , Epilepsia/dietoterapia , Doenças do Sistema Nervoso/dietoterapia , Dieta Cetogênica/efeitos adversos , Epilepsia/metabolismo , Seguimentos , Humanos , Doenças do Sistema Nervoso/metabolismo , Resultado do TratamentoRESUMO
PURPOSE: This study examines electroencephalographic (EEG) changes in children with medication resistant epilepsy treated with the ketogenic diet (KD). METHODS: Routine EEGs were obtained prior to KD initiation, then one month and three months later. Changes in EEG background slowing and frequency of interictal epileptiform discharges (IEDs) were evaluated using power spectrum analysis and manual determination of spike index. KD responders were compared to non-responders to determine if baseline or early EEG characteristics predicted treatment response (>50% seizure reduction) at three months. RESULTS: Thirty-seven patients were evaluated. No differences in baseline EEG features were found between responder groups. Frequency of IEDs declined in 65% of patients as early as one month, by a median of 13.6% (IQR 2-33). Those with a ten percent or greater improvement in IED frequency at one month were greater than six times more likely to be KD responders (OR 6.5 95% CI 0.85-75 p=0.03). Qualitative and quantitative measures of EEG background slowing improved in the whole cohort, but did not predict responder status. CONCLUSION: Baseline predictors of KD response remain elusive. Most patients experienced a reduction in IEDs and improvement in EEG background slowing after KD initiation. Reduction of IEDs at one month strongly predicted KD responder status at three months.
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Dieta Cetogênica/métodos , Eletroencefalografia , Epilepsia/dietoterapia , Adolescente , Criança , Pré-Escolar , Eletroencefalografia/métodos , Epilepsia/diagnóstico , Feminino , Humanos , Lactente , Masculino , Estatísticas não ParamétricasRESUMO
BACKGROUND: The ketogenic diet (KD) is a high-fat, low-carbohydrate, and protein diet that effectively treats intractable epilepsy (IE). OBJECTIVE: The purpose of this study was to measure the change in bone mineral content (BMC) in children with IE treated with the KD for 15 mo. DESIGN: Prepubertal children >or=5 y of age with IE were eligible. A 4:1 ketogenic diet was maintained for 15 mo, and whole-body and spine BMCs were measured with dual-energy X-ray absorptiometry. Z scores were generated by comparing the children with IE with a cohort of 847 healthy children. Other measurements included demographics, anthropometry, serum 25-hydroxyvitamin D (25-OHD), intact parathyroid hormone, electrolytes, and dietary intake. All measurements were performed at baseline and at 3, 6, 12, and 15 mo. Longitudinal mixed effects models were used to analyze change in BMC over time. RESULTS: Twenty-five children (9 girls, 16 boys) with IE [age (x +/- SD): 7.3 +/- 1.9 y] participated. Growth and bone health status were suboptimal as were serum 25-OHD concentrations and dietary intake of calcium and vitamin D. Whole-body and spine BMC-for-age both declined by 0.6 z score/y and whole-body and spine BMC-for-height declined 0.7 z score/y and 0.4 z score/y, respectively. Height declined 0.5 z score/y. Body mass index (BMI; in kg/m(2)) z score, age, and ambulation were positive predictors of BMC, which declined sharply over 15 mo of KD treatment. CONCLUSION: Bone health in children with IE was poor, particularly for younger nonambulatory children with low BMI status. The KD resulted in progressive loss of BMC. The mechanism is unclear. Further studies are needed.
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Densidade Óssea , Osso e Ossos/metabolismo , Dieta Cetogênica/efeitos adversos , Epilepsia/dietoterapia , Cetose/metabolismo , 25-Hidroxivitamina D 2/sangue , Absorciometria de Fóton/métodos , Índice de Massa Corporal , Densidade Óssea/efeitos dos fármacos , Densidade Óssea/fisiologia , Cálcio/sangue , Cálcio da Dieta/administração & dosagem , Cálcio da Dieta/metabolismo , Criança , Epilepsia/complicações , Feminino , Humanos , Estudos Longitudinais , Masculino , Estado Nutricional , Osteoporose/epidemiologia , Osteoporose/etiologia , Vitamina D/administração & dosagem , Vitamina D/sangueRESUMO
The aim of this study was to evaluate the resting energy expenditure (REE) of children with intractable epilepsy (IE) compared with healthy children, and to determine factors that contribute to the pattern of REE. REE, growth status, and body composition were assessed in 25 prepubertal children with IE (15 males, 10 females; mean age 5y 5mo [SD 2y 2mo] range 2-9y) with and without cerebral palsy (CP) and compared with those in 75 healthy children of similar age, sex, and fat free mass (FFM; 43 males, 32 females; mean age 6y 4mo [SD 1y 8mo], range 2-9y). Of the 25 children with IE, 12 had generalized and 13 partial seizures; 10 children had CP (four hemiplegia, one diplegia, and five tetraplegia); 18 were ambulators. REE (kcal/d), determined by indirect calorimetry, was expressed as a percentage of that predicted using Schofield equations. Energy intake from 3-day weighed food records was assessed for children with IE only and expressed as a percentage of estimated energy requirement. Compared with healthy children, children with IE had significantly lower percentage (Student's t-test, p<0.05) of predicted REE (111 [SD 13] vs 104 [SD 4]), weight z-score, body mass index z-score, and FFM. Using multiple regression, REE adjusted for FFM, fat mass, and sex were significantly lower in children with IE and CP (-110 kcal/d, 95% confidence interval -199 to -21, p=0.016). In children with IE, energy intake was also a statistically significant predictor of REE. CP largely explained the suboptimal growth status and lower REE of children with IE compared with healthy children.
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Ingestão de Energia , Metabolismo Energético , Epilepsia/epidemiologia , Transtornos do Crescimento/epidemiologia , Índice de Massa Corporal , Calorimetria , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Prospectivos , DescansoRESUMO
PURPOSE: Myoclonic astatic epilepsy (MAE) is a generalized epilepsy of early childhood. Little is known about the use of newer antiepileptic treatments (AET) in MAE. The purpose of this study was to describe the characteristics, treatment, and outcome of a contemporary MAE cohort exposed to the new generation AET. METHODS: Charts of subjects with MAE treated between 1998 and 2005 were reviewed. RESULTS: Twenty-three subjects (19 boys), with a median (range) follow-up of 38 (2- 86) months were identified. Thirty-nine percent had a family history of epilepsy, and 39% had family history of febrile seizures. Age at seizure onset was a median of 36 (12-24) months. Initial EEG was normal in 30%. When seizures ceased, EEG background and epileptiform abnormalities persisted in 17 and 58%, respectively. On average, each subject was exposed to five AET. The most frequently used AET was valproate (83%). Seizure freedom occurred spontaneously in three subjects, with ethosuximide and levetiracetam in one each, valproate and lamotrigine in two each, topiramate in three and the ketogenic diet (KD) in five subjects. By 36 months after seizure onset, 67% achieved seizure freedom. At the last visit, 43% were developmentally normal, 52% had mild, and 5% had moderate cognitive disabilities. Time to seizure freedom did not correlate with cognitive outcome. CONCLUSIONS: The new generation of AET may offer significant benefit to children with MAE. The KD was the most effective AET in this series, and perhaps should be considered earlier in treatment.
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Anticonvulsivantes/uso terapêutico , Epilepsias Mioclônicas/terapia , Cetose/induzido quimicamente , Cetose/metabolismo , Idade de Início , Criança , Transtornos Cognitivos/tratamento farmacológico , Transtornos Cognitivos/terapia , Gorduras na Dieta/administração & dosagem , Gorduras na Dieta/metabolismo , Eletroencefalografia/estatística & dados numéricos , Epilepsias Mioclônicas/dietoterapia , Epilepsias Mioclônicas/tratamento farmacológico , Etossuximida/uso terapêutico , Feminino , Hospitais Pediátricos , Humanos , Cetonas/metabolismo , Lamotrigina , Levetiracetam , Masculino , Pennsylvania , Piracetam/análogos & derivados , Piracetam/uso terapêutico , Estudos Retrospectivos , Análise de Sobrevida , Síndrome , Triazinas/uso terapêuticoRESUMO
Growth retardation is common among children with epilepsy, and poor dietary intake may be one of the causes. The goal of this cross-sectional study was to compare the nutrient intake of children 1 to 8 years of age with intractable epilepsy to healthy children of the same age from the National Health and Nutrition Examination Survey 2001 to 2002 (N=1,718) and with the Dietary Reference Intakes. Children with intractable epilepsy were divided into two age groups: 1.0 to 3.9 and 4.0 to 8.9 years, to correspond with the Dietary Reference Intakes. Forty-three children with intractable epilepsy, mean age=4.7+/-2.2 years, had significantly lower intakes (P<0.05) of total energy; protein; carbohydrate; fat; dietary fiber; vitamins A, E, B-6, and B-12; riboflavin; niacin; folate; calcium; phosphorus; magnesium; zinc; copper; and selenium compared with healthy children. Thirty percent or more of the children with intractable epilepsy in both age groups had intakes below the Recommended Dietary Allowance or Adequate Intake for vitamins D, E, and K; folate; calcium; linoleic acid; and alpha-linolenic acid. Health care professionals caring for children with intractable epilepsy should be aware of this pattern of decreased nutrient intake and educate families to provide an adequate diet and/or consider vitamin/mineral supplementation.
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Transtornos da Nutrição Infantil/etiologia , Fenômenos Fisiológicos da Nutrição Infantil/fisiologia , Ingestão de Energia/fisiologia , Epilepsia/fisiopatologia , Inquéritos Nutricionais , Estudos de Casos e Controles , Criança , Transtornos da Nutrição Infantil/epidemiologia , Pré-Escolar , Carboidratos da Dieta/administração & dosagem , Gorduras na Dieta/administração & dosagem , Proteínas Alimentares/administração & dosagem , Feminino , Humanos , Lactente , Masculino , Minerais/administração & dosagem , Política Nutricional , Necessidades Nutricionais , Vitaminas/administração & dosagemRESUMO
PURPOSE: The aim of this study was to describe vitamin D status in children with intractable epilepsy prescribed newer antiepileptic drugs (AEDs) before initiation of and during 15-month treatment with the ketogenic diet (KD). METHODS: Serum vitamin D (25-OHD and 1,25-OHD) and parathyroid hormone (PTH) were assessed in prepubertal children with intractable epilepsy before initiation of and during KD therapy. Three-day weighed dietary records including KD and vitamin and mineral supplementation were obtained at baseline and at 1 month. RESULTS: Forty-five children (aged 5.1 +/- 2.7 years) were enrolled. Before KD therapy, 4% had deficient and 51% had insufficient serum 25-OHD levels. Vitamin D intake was less than recommended in 47%. Adequate vitamin D intake, fewer AEDs, and generalized seizures were associated with higher serum 25-OHD levels (p < 0.01). After 3 months on the KD, 25-OHD levels increased (p < 0.001), and PTH declined (p < 0.001). Over the next 12-month period, 25-OHD levels steadily declined (p < 0.001), and PTH did not significantly change. CONCLUSIONS: Children with intractable epilepsy treated with newer AEDs had poor vitamin D status. Their status improved over the first 3 months of KD therapy with vitamin D supplementation and slowly declined thereafter.
Assuntos
Anticonvulsivantes/efeitos adversos , Gorduras na Dieta/administração & dosagem , Epilepsia/sangue , Epilepsia/dietoterapia , Estado Nutricional , Deficiência de Vitamina D/sangue , Vitamina D/sangue , 25-Hidroxivitamina D 2/sangue , 25-Hidroxivitamina D 2/deficiência , Adolescente , Criança , Pré-Escolar , Gorduras na Dieta/metabolismo , Suplementos Nutricionais , Epilepsia/tratamento farmacológico , Feminino , Humanos , Lactente , Cetose/metabolismo , Estudos Longitudinais , Masculino , Hormônio Paratireóideo/sangue , Estações do Ano , Deficiência de Vitamina D/induzido quimicamenteRESUMO
OBJECTIVE: The concept of "rational polypharmacy" has been associated with anticonvulsant management for decades, but the term has not been applied to nonpharmacologic therapies. METHODS: We conducted a multicenter, retrospective study of children who received concurrent diet (ketogenic or modified Atkins) and vagus nerve stimulation (VNS) treatment for medically intractable epilepsy. RESULTS: Thirty children in total from six epilepsy centers were treated over a 6-yr period. The median age at the initiation of combination therapy was 10 yr (range, 4-24 yr). Sixteen (53%) received dietary therapy followed by VNS; no differences were noted between centers. After 3 months, 21 (70%) had seizure reduced by >50% over the previous single nonpharmacologic treatment, of whom 13 (62%) had improvement within the first month. A 5-min VNS off-time correlated with >90% seizure reduction (p = 0.02). The median duration of nonpharmacologic polytherapy was 12 months (range, 0.5-96 months); 17 (57%) remain on dual therapy at this time. No side effects were noted. Most patients who discontinued combination therapy did so because of a lack of efficacy rather than restrictiveness. CONCLUSIONS: In this small group, the combined use of diet and VNS appeared synergistic and yielded rapid benefits. It may be more effective with longer VNS off-times. Further prospective studies of this combination in refractory pediatric epilepsy are needed to help guide optimal use.
Assuntos
Gorduras na Dieta/administração & dosagem , Gorduras na Dieta/metabolismo , Terapia por Estimulação Elétrica/métodos , Epilepsia/dietoterapia , Epilepsia/terapia , Cetose/metabolismo , Nervo Vago/fisiologia , Adolescente , Adulto , Criança , Pré-Escolar , Terapia Combinada , Dieta com Restrição de Carboidratos , Epilepsia/metabolismo , Estudos de Avaliação como Assunto , Feminino , Humanos , Estudos Longitudinais , Masculino , Estudos Retrospectivos , Resultado do TratamentoRESUMO
PURPOSE: The ketogenic diet (KD) is a 90% fat diet that is an effective treatment for intractable epilepsy. Rapid initiation of the KD requires hospital admission because of the complexity of the protocol and frequent mild and moderate adverse events. The purpose of the study was to compare the efficacy of a gradual KD initiation with the standard KD initiation preceded by a 24- to 48-h fast. METHODS: Children ages 1 to 14 years with intractable epilepsy were randomized to a fasting initiation (FAST-KD) or gradual initiation (GRAD-KD). Baseline seizure activity was recorded daily for 28 days before admission and continued for the 3-month duration of the study. Effectiveness was measured in two ways: (a) the proportion of subjects with >50% reduction in target seizure type from baseline to 3-month evaluation, and (b) percentage reduction in the frequency of the target seizure type from baseline to 3-month evaluation. Blood glucose was assessed q4 to 6h, and weights, electrolytes, hydration status, vomiting, acid balance, need for interventions (citric acid and sodium citrates (Bicitra) and IV fluids) were assessed daily. Fisher's exact tests were used to examine the association between protocol and occurrence of adverse events, and longitudinal mixed-effects models were used to look for trends in tolerability data over time. RESULTS: Forty-eight subjects, 24 in each arm, were randomized. In the FAST-KD protocol, 58% of the children had >50% reduction in the target seizure type at 3 months, and 21% were seizure free. In the GRAD-KD protocol, 67% had a >50% reduction at 3 months, and 21% were seizure free. The two protocols were equivalent in efficacy (p = 0.033). At 3 months, the FAST-KD median percentage seizure reduction rate was 78% (ranging from 100% reduction to 73% increase in seizures per week) and was 94% (ranging from 100% reduction to 161% increase in seizures per week) for the GRAD-KD protocol. By using a logarithmic transformed percentage reduction rate and an equivalence limit difference of 20%, the efficacy of the two protocols was equivalent (p = 0.0002). Children in the GRAD protocol lost significantly less weight (p = 0.006), and had fewer and less-severe episodes of hypoglycemia (p < 0.001), fewer treatments for acidosis (citric acid and sodium citrates) (p < 0.04) and dehydration (IV fluids) (p < 0.04), but no difference in vomiting was noted. CONCLUSIONS: These data suggest that in children with intractable epilepsy, a gradual initiation results in fewer adverse events and is tolerated better overall while maintaining the efficacy of the KD.