In this paper, we report on a 5-year-old girl who developed a renal stone while following the ketogenic diet to treat refractory seizure disorder. Three months after initiating the ketogenic diet, she developed severe abdominal pain and vomiting. The spot urine calcium-to-creatinine (Ca/Cr) ratio and 24-hour urine evaluation showed hypercalciuria. Computed tomography (CT) imaging revealed a stone in the right ureteropelvic junction, resulting in hydronephrosis of the right kidney. The renal stone disappeared 5 days after conservative treatment; the patien's microscopic hematuria resolved concurrently. In light of this case report, we recommend regularly monitoring the urine Ca/Cr ratio with ultrasonography for further development of renal stones in patients following the ketogenic diet. If these patients exhibit evidence of symptomatic hypercalciuria or cyristalluria, liberalization of fluid restriction and urine alkalization using oral potassium citrate should be considered.
Child, Preschool ; Epilepsy/*diet therapy ; Female ; Humans ; Ketogenic Diet/*adverse effects/methods ; Kidney Calculi/*etiology

OBJECTIVETo study the clinical efficiency, electroencephalogram (EEG) changes and cognitive improvements of ketogenic diet (KD) in children with refractory epilepsy.

METHODSTwenty pediatric patients (7-61 months in age) with refractory epilepsy were recruited between August 2012 and August 2013. KD therapy was performed on all participants for at least 3 months based on a fasting initiation protocol with the lipid-to-nonlipid ratio being gradually increased to 4 : 1. Seizure frequency, type and degree were recorded before and during KD therapy. A 24 hours video-electroencephalogram (V-EEG) examination and Gesell Developmental Scale assessment were performed prior to KD therapy, and 3, 6, 9 months after KD therapy.

RESULTSSix patients became seizure free after KD therapy, with a complete control rate of 30%. Seizure frequency reduction occurred in 13 (65%) patients, EEG improvement in 8 (40%) patients, and improvement in Gesell Developmental Scales (gross motor and adaptability in particular) in 6 (30%) patients. The KD therapy-related side effects were mild.

CONCLUSIONSKD therapy is safety and effective in reducing seizure frequency and improving EEG and cognitive function in children with refractory epilepsy.

Child, Preschool ; Diet, Ketogenic ; adverse effects ; Electroencephalography ; Epilepsy ; diet therapy ; physiopathology ; Female ; Humans ; Infant ; Male ; Prospective Studies ; Recurrence

Epilepsy ; diet therapy ; metabolism ; Food-Drug Interactions ; Humans ; Ketone Bodies ; biosynthesis

Adenosine ; metabolism ; Adenosine Kinase ; metabolism ; Adenosine Triphosphate ; metabolism ; Animals ; Brain ; drug effects ; metabolism ; Diet, Carbohydrate-Restricted ; methods ; Diet, Ketogenic ; Energy Metabolism ; Epilepsy ; diet therapy ; metabolism ; Humans

Epilepsy is one of the most common and disabling neurologic disorders in childhood. The primary goal of epilepsy treatment is to choose the treatment modality that provides the best chance of improving the patient's quality of life. In addition to classic antiepileptic drugs, newly developed antiepileptic drugs ketogenic diet, epilepsy surgery, and vagal nerve stimulation have improved the ability to treat seizure disorders during the past decades. The mainstay of treatment remains pharmacotherapy. The factors that affect decision of whether to treat seizures with medications and which antiepileptic drugs to use include the likelihood of seizure recurrence, type and severity of seizures, available antiepileptic drug efficacies and toxicities, need for hematologic monitoring, ease to dosing, underlying illness, pharmacologic interaction, and school and other social issues. In this article, we will describe the principle of drug selection for seizure disorder in pediatric patients and the efficacies and adverse effects of the typical and new antiepileptics. Information about the available antiepileptic drugs will help the pediatrician to select the best drug for children with epilepsy.
Anticonvulsants ; Child ; Drug Therapy* ; Epilepsy* ; Humans ; Ketogenic Diet ; Nervous System Diseases ; Quality of Life ; Recurrence ; Seizures ; Vagus Nerve Stimulation

The ketogenic diet is an effective treatment for the patients with intractable epilepsy, however, the diet therapy can sometimes be discontinued by complications. Protein–losing enteropathy is a rarely reported serious complication of the ketogenic diet. We present a 16-month-old Down syndrome baby with protein-losing enteropathy during the ketogenic diet as a treatment for West syndrome. He suffered from diarrhea, general edema and hypoalbuminemia which were not controlled by conservative care for over 1 month. Esophagogastroduodenoscopy and stool alpha-1 antitrypsin indicated protein-losing enteropathy. Related symptoms were relieved after cessation of the ketogenic diet. Unexplained hypoalbuminemia combined with edema and diarrhea during ketogenic suggests the possibility of protein-losing enteropathy, and proper evaluation is recommended in order to expeditiously detect it and to act accordingly.
Diarrhea ; Diet Therapy ; Down Syndrome ; Drug Resistant Epilepsy ; Edema ; Endoscopy, Digestive System ; Humans ; Hypoalbuminemia ; Infant ; Infant, Newborn ; Ketogenic Diet* ; Protein-Losing Enteropathies* ; Spasms, Infantile

The ketogenic diet is a high-fat, low-protein, low-carbohydrate diet developed in the 1920s for the treatment of difficult-to-control seizures. Despite advances in both the pharmacotherapy and the surgery of epilepsy, many children continue to have difficult-to-control seizures. In this situation, a ketogenic diet should be considered as an alternative therapy. However, less attention has been paid to associated adverse events in the ketogenic diet. We report a case of infantile spasm associated with acute renal failure, lipoid pneumonitis and kwashiorkor after ketogenic diet. A better understanding of this adverse event profile will allow the pediatric neurologist to have a true informed consent discussion with the care giver when considering initiation of the ketogenic diet.
Acute Kidney Injury* ; Caregivers ; Child ; Diet ; Drug Therapy ; Epilepsy ; Humans ; Infant ; Infant, Newborn ; Informed Consent ; Ketogenic Diet* ; Kwashiorkor* ; Pneumonia ; Seizures ; Spasms, Infantile*

OBJECTIVETo investigate the effect of ketogenic diet (KD) on the clinical and electroencephalogram features in children with pharmacoresistant epileptic encephalopathy.

METHODThirty-one children (19 boys, 12 girls) aged 7 months to 7 years (mean 2 years 5 month) with epilepsy refractory to conventional antiepileptic drugs (AEDs) were included in this study. In addition to their original AED treatment, the children were assigned to different ketogenic diets based on their age. The prospective electro-clinical assessment was performed prior to the KD and then one week, one month and again 3 months after the initiation of therapy, respectively.

RESULTThe reduction of seizure frequency in 52%, 68% and 71% of all patients exceeded 50% one week, one month and three months after KD treatment respectively. KD is particularly effective in myoclonic astatic epilepsy (MAE; Doose Syndrome) and West syndrome with 100% and 81.25% of the patients having a greater than 50% seizure reduction, respectively. After 3 months of KD treatment, more than 2/3 patients experienced a reduction in interictal epileptiform discharges (IEDs) and improvement in EEG background.

CONCLUSIONThe clinical and electroencephalographic improvement confirms that KD is beneficial in children with refractory epilepsy.

Anticonvulsants ; therapeutic use ; Brain ; diagnostic imaging ; physiopathology ; Child ; Child, Preschool ; Diet, Ketogenic ; methods ; Dietary Fats ; administration & dosage ; Electroencephalography ; Epilepsy ; diagnosis ; diet therapy ; drug therapy ; Female ; Humans ; Infant ; Intellectual Disability ; diet therapy ; drug therapy ; Lennox Gastaut Syndrome ; Male ; Radiography ; Retrospective Studies ; Spasms, Infantile ; diet therapy ; drug therapy ; Syndrome ; Time Factors ; Treatment Outcome

Studies of epilepsy have mainly focused on the membrane proteins that control neuronal excitability. Recently, attention has been shifting to intracellular proteins and their interactions, signaling cascades and feedback regulation as they relate to epilepsy. The mTOR (mammalian target of rapamycin) signal transduction pathway, especially, has been suggested to play an important role in this regard. These pathways are involved in major physiological processes as well as in numerous pathological conditions. Here, involvement of the mTOR pathway in epilepsy will be reviewed by presenting; an overview of the pathway, a brief description of key signaling molecules, a summary of independent reports and possible implications of abnormalities of those molecules in epilepsy, a discussion of the lack of experimental data, and questions raised for the understanding its epileptogenic mechanism.
Astrocytes/metabolism ; Cell Death ; Epilepsy/diet therapy/drug therapy/*metabolism/virology ; Humans ; Ketogenic Diet ; Protein Binding/physiology ; Protein Kinase Inhibitors/therapeutic use ; Receptors, Cannabinoid/metabolism ; Signal Transduction/*physiology ; Synapses/metabolism ; TOR Serine-Threonine Kinases/antagonists & inhibitors/*metabolism ; Temporal Lobe/metabolism

The purpose of this article is to describe the overview of current medical treatments of childhood epilepsy in Korea and to review several recent nursing researches related to quality of life problems, especially psychological functioning in children with epilepsy and the stress of the family. The prognosis of childhood epilepsy has been improved considerably and about 80% of patients can now be expected to achieve complete seizure control by the antiepileptic drug treatment. Even for the intractable epilepsy, with the combination of ketogenic diet program and antiepileptic drug therapy or surgical treatment, the prognosis became very much better than before. The majority of research has reported that children with epilepsy were experiencing quality of life problems. They are at risk for impaired functioning, compared to either general population controls or to other chronic illness groups such as asthma and diabetes. The ultimate goal of providing care to children with epilepsy is to control seizures while facilitating an optimal quality of life for the child as well as the family. Recommendations are included for future research and intervention programs for children, parents and our society.
Asthma ; Child* ; Chronic Disease ; Drug Therapy ; Epilepsy* ; Humans ; Ketogenic Diet ; Korea ; Nursing Research ; Parents ; Population Control ; Prognosis ; Quality of Life* ; Seizures ; Child Health