ABSTRACT
The ketogenic diet is a high-fat, low carbohydrate, adequate protein diet, developed in the 1920s for the management of intractable seizure disorders in children. To evaluate efficacy and tolerability of the classic ketogenic diet, we analyzed records of the children started on the diet from 1999 to 2006 at the Mofid children's hospital. The subjects were 87 children, mean age 55 months. Before initiation of the diet, 55% of the patients had seizures, at least 1-4 times per day, 36%-5 or more per day and 9%-2 to 4 times per week. Mean number of Anti Epileptic Drugs [AEDs] tried for them was 8 and 67% were receiving three or more drugs. The ketogenic diet showed drastic improvement, with at least 50% reduction in seizure frequency in 87% of our patients, 39% of whom showed complete seizure control in the third month. After one year, in 80% of the patients who returned, improvement continued, with 26% of them being seizure free; besides, 23% had one AED decreased, 36% had two or three AEDs decreased, and 25% [one child] had all AEDs discontinued. Of the 30 improved cases, 20%, at the end of the first year, had improved behavior as well, and 23% of them had become more alert. The medium diet duration of the improved group was 15 months. The improvement in our patients, low side effects, and the duration of diet by families reveal that the ketognic diet can still be a very useful alternative therapy in certain epileptic children
Subject(s)
Humans , Male , Female , Seizures/epidemiology , Seizures/diet therapy , Child , Treatment Outcome , Epilepsy , AnticonvulsantsABSTRACT
Background: Ketogenic diet (KD) represents an alternative in treatment of refractory epilepsy (RE). Objective: To evaluate the efficacy of the diet and the frequency of complications in patients belonging to the KD Program from Luis Calvo Mackenna Children's Hospital (HLCM). Methods: Evaluation of all children enrolled in the program between 1999 and 2004, with analysis every 6 months of the diet efficacy, digestive tolerance, nutritional status, cholesterol levels and nephrolithiasis. Results: 21 children were admitted, 14 boys, age between 6 months - 17 years-old. 76 percent, 71 percent and 67 percent of patients followed KD at 6, 12 and 18 months, respectively, with KD efficacy of 67 percent. At 12 months, 24 percent of patients did not present seizures. At 18 months, 85 percent remained close to ideal body weight (15 percent obesity) and height/age Z score decreased (-0,7 +/- 0,4; p < 0,05). Total cholesterol significantly increased at 6 months (64 percent hypercholesterolemia; decreased to 15 percent at 18 months). 2 patients developed nephrolithiasis. Conclusions: The study shows high efficacy of the KD for treatment of refractory epilepsy, with low rate of complications. It should be considered as a therapeutic alternative for these patients.
La dieta cetogénica (DK) es una opción de tratamiento en epilepsia refractaria (ER). En Chile no hay estudios publicados al respecto. Objetivo: Evaluar la eficacia de la dieta en el control de las convulsiones y la frecuencia de complicaciones en los pacientes del programa de DK, para el tratamiento de ER, del Hospital Luis Calvo Mackena (HLCM). Pacientes y Método: Evaluamos todos los niños ingresados al programa entre 1999-2004. Para efectos de este trabajo se consideró el control al ingreso y cada 6 meses, evaluándose: eficacia de la dieta, tolerancia digestiva, evolución nutricional, niveles de colesterol plasmático y litiasis renal. Resultados: Ingresaron 21 niños de 6,2 años (6 meses a 17 años), 14 de sexo masculino. A los 6, 12 y 18 meses, 76 por ciento, 71 por ciento y 67 por ciento de los pacientes, respectivamente, se mantenía en dieta. La eficacia del tratamiento fue 67 por ciento. A los 12 meses, 24 por ciento de los pacientes estaba sin crisis. A los 18 meses 85 por ciento de los pacientes estaba eutrófico y 15 por ciento obeso. Se observó deterioro en la talla (delta zT/E -0,7 +/- 0,4; p < 0,05). El colesterol total aumentó significativamente a los 6 meses, encontrándose el 64 por ciento hipercolesterolémico; a los 18 meses este porcentaje se redujo a 15 por ciento. Dos pacientes presentaron litiasis renal (9 por ciento). Conclusiones: Este estudio muestra una muy buena eficacia de la dieta cetogénica para el tratamiento de la epilepsia refractaria, y una baja frecuencia de complicaciones, por lo que debería ser considerada como alternativa terapéutica en estos pacientes.
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Epilepsy/diet therapy , Dietary Fats/therapeutic use , Anticonvulsants/therapeutic use , Kidney Calculi/etiology , Ketosis/metabolism , Cholesterol/blood , Seizures/diet therapy , Ketone Bodies/biosynthesis , Epilepsy/metabolism , Epilepsy/blood , Follow-Up Studies , Dietary Fats/adverse effects , Time Factors , Treatment OutcomeABSTRACT
The glucose transporter type 1 deficiency syndrome (GLUT-1 SD) (OMIM 606777) is an inborn error of metabolism of brain glucose transport. The characteristic clinical manifestations are seizures, hypotonia, developmental delay, microcephaly and hypoglycorrhachia. We report a girl with normal weight and height at birth. At 6 weeks of age she started with convulsions reaching up to 20 myoclonic seizures a day. She was treated with valproate, phenobarbital and carbamazepine without response. Blood analysis including aminoacids and acylcarnitines were all normal. The brain MRI showed frontal atrophy with an increased subarachnoidal space and Electroencephalography was abnormal. Blood glucose was 84 mg/dl and spinal fluid glucose 26 mg/dl with a ratio of 0.31 (Normal Ratio >0.65+00.1). These results suggested the diagnosis of GLUT-1 SD, and was confirmed with erythrocyte glucose uptake of 44 percent (Normal range 80-100 percent). A molecular study found the mutation 969del, C971T in exon 6 of the gene Glut-1. Treatment with a ketogenic diet was started immediately and after 7 days with this diet seizures ceased. Anticonvulsants were progressively suspended. At present, the patient is 6 years old, she continues on a ketogenic diet and supplements with L-carnitine, lipoic acid, vitamins and minerals. Growth and development are normal with an intelligence quotient of 103. It is concluded that it is necessary to include GLUT-1 SD in the differential diagnosis of children with early seizures that are non responsive to pharmacological treatment.