Pseudotumor cerebral asociado a hipovitaminosis A, B6 y D: A propósito de dos casos / Pseudotumor cerebri associated with hypovitaminosis A, B6 and D: About two cases

La hipertensión endocraneana idiopàtica se asocia infrecuentemente con la hipovitaminosis A y D. Se presenta el caso de una paciente femenina de 8 años con visión borrosa de 24 horas y papiledema bilateral. Resonancia magnética nuclear normal. Presión de apertura de líquido cefalorraquídeo: 260 mm^O. Presentó déficit de vitamina A y D, e inició un tratamiento sustitutivo. El segundo caso corresponde a un paciente masculino de 12 años con fiebre y odinofagia de 3 días. Con antecedente de glomerulonefritis y sobrepeso. Presentaba edema bipalpebral y papiledema. Tomografia axial computada de la órbita: aumento de líquido en la vaina de ambos nervios ópticos. Resonancia magnética nuclear: aracnoidocele intraselar. Presión de apertura de líquido cefalorraquídeo: 400 mm^O. Presentó déficit de vitamina D y B6, e inició el tratamiento sustitutivo. La elevación de la presión intracraneal desencadena mecanismos de compensación que, al fallar, pueden comprometer la vida o provocar graves discapacidades neurológicas. Reconocer la causa para un enfoque terapéutico preciso es clave para disminuir la morbimortalidad asociada a esta patología.

Idiopathic endocranial hypertension is infrequently associated with hypovitaminosis A and D. The case of an 8-year-old female with 24-hour blurred vision and bilateral papilledema is presented. Nuclear magnetic resonance was normal. Opening pressure of cerebrospinal fluid: 260 mm^O. She presented vitamin A and D deficiency and started replacement therapy. The second case corresponds to a 12-year-old male with fever and odynophagia of 3 days. History of glomerulonephritis and overweight. He had bipalpebral edema and papilledema. Computed tomography scan of the orbit: increase of fluid in the sheath of both optic nerves. Nuclear magnetic resonance: intrasellar arachnoidocele. Opening pressure of cerebrospinal fluid: 400 mmH2O. He presented vitamin D and B6 deficiency and started replacement treatment. The elevation of intracranial pressure triggers compensation mechanisms that, when they fail, can compromise life or cause serious neurological disabilities. Recognizing the cause for an accurate therapeutic approach is key to reduce the morbidity and mortality associated with this pathology.

[Pseudotumor cerebri associated with hypovitaminosis A, B6 and D. About two cases]. / Pseudotumor cerebral asociado a hipovitaminosis A, B6 y D. A propósito de dos casos.

Idiopathic endocranial hypertension is infrequently associated with hypovitaminosis A and D. The case of an 8-year-old female with 24-hour blurred vision and bilateral papilledema is presented. Nuclear magnetic resonance was normal. Opening pressure of cerebrospinal fluid: 260 mmH2O. She presented vitamin A and D deficiency and started replacement therapy. The second case corresponds to a 12-year-old male with fever and odynophagia of 3 days. History of glomerulonephritis and overweight. He had bipalpebral edema and papilledema. Computed tomography scan of the orbit: increase of fluid in the sheath of both optic nerves. Nuclear magnetic resonance: intrasellar arachnoidocele. Opening pressure of cerebrospinal fluid: 400 mmH2O. He presented vitamin D and B6 deficiency and started replacement treatment. The elevation of intracranial pressure triggers compensation mechanisms that, when they fail, can compromise life or cause serious neurological disabilities. Recognizing the cause for an accurate therapeutic approach is key to reduce the morbidity and mortality associated with this pathology.

La hipertensión endocraneana idiopática se asocia infrecuentemente con la hipovitaminosis A y D. Se presenta el caso de una paciente femenina de 8 años con visión borrosa de 24 horas y papiledema bilateral. Resonancia magnética nuclear normal. Presión de apertura de líquido cefalorraquídeo: 260 mmH2O. Presentó déficit de vitamina A y D, e inició un tratamiento sustitutivo. El segundo caso corresponde a un paciente masculino de 12 años con fiebre y odinofagia de 3 días. Con antecedente de glomerulonefritis y sobrepeso. Presentaba edema bipalpebral y papiledema. Tomografía axial computada de la órbita: aumento de líquido en la vaina de ambos nervios ópticos. Resonancia magnética nuclear: aracnoidocele intraselar. Presión de apertura de líquido cefalorraquídeo: 400 mmH2O. Presentó déficit de vitamina D y B6, e inició el tratamiento sustitutivo. La elevación de la presión intracraneal desencadena mecanismos de compensación que, al fallar, pueden comprometer la vida o provocar graves discapacidades neurológicas. Reconocer la causa para un enfoque terapéutico preciso es clave para disminuir la morbimortalidad asociada a esta patología.

[National consensus on the modified Atkins diet]. / Consenso nacional de dieta Atkins modificada.

INTRODUCTION: Epilepsy is a chronic disease that affects 0.5-1% of the population. One third of the patients become refractory to antiepileptic drugs. Among the non-pharmacological treatments available, the modified Atkins diet is an effective treatment used since 2003 as another alternative for children and adults with refractory epilepsy. DEVELOPMENT: The Ketogenic Diet National Committee, which depends on the Argentine Society of Pediatric Neurology, elaborated this consensus on the modified Atkins diet, basing itself on a review of the literature and on their clinical experience. This consensus in Spanish explains the different aspects to be taken into account regarding the modified Atkins diet, patient selection, implementation, different controls and adverse effects. Unlike the classic ketogenic diet, the modified Atkins diet is initiated without fasting or hospital stay, nor does it require protein, calorie or fluid restriction, thus improving patient palatability and consequently patient tolerability. CONCLUSIONS: The modified Atkins diet is a useful treatment for patients with intractable epilepsy. The publication of this consensus offers the possibility for new centers to get oriented regarding this diet implementation.

TITLE: Consenso nacional de dieta Atkins modificada.Introduccion. La epilepsia es una enfermedad cronica que afecta al 0,5-1% de la poblacion, y un tercio de los pacientes evoluciona hacia una forma refractaria a los farmacos antiepilepticos. Dentro de los tratamientos no farmacologicos disponibles, la dieta cetogenica Atkins modificada es un tratamiento efectivo utilizado desde 2003 como otra alternativa en niños y adultos con epilepsia refractaria. Desarrollo. El Comite Nacional de Dieta Cetogenica, dependiente de la Sociedad Argentina de Neurologia Infantil, elaboro este consenso sobre dieta Atkins modificada basandose en una revision de la bibliografia y en su experiencia clinica. Este consenso explica los distintos aspectos que hay que tener en cuenta sobre la dieta Atkins modificada, eleccion de pacientes, forma de implementacion, diversos controles y efectos adversos. A diferencia de la dieta cetogenica clasica, se inicia sin ayuno ni hospitalizacion, y no hay restriccion proteica, calorica o hidrica, por lo que mejora la palatabilidad y, consecuentemente, la tolerabilidad. Conclusiones. La dieta Atkins modificada es un tratamiento util para pacientes con epilepsia intratable. La publicacion de este consenso ofrece la posibilidad de orientar a nuevos centros en su implementacion.

Consenso nacional de dieta Atkins modificada / National consensus on the modified Atkins diet

Introducción. La epilepsia es una enfermedad crónica que afecta al 0,5-1% de la población, y un tercio de los pacientes evoluciona hacia una forma refractaria a los fármacos antiepilépticos. Dentro de los tratamientos no farmacológicos disponibles, la dieta cetogénica Atkins modificada es un tratamiento efectivo utilizado desde 2003 como otra alternativa en niños y adultos con epilepsia refractaria. Desarrollo. El Comité Nacional de Dieta Cetogénica, dependiente de la Sociedad Argentina de Neurología Infantil, elaboró este consenso sobre dieta Atkins modificada basándose en una revisión de la bibliografía y en su experiencia clínica. Este consenso explica los distintos aspectos que hay que tener en cuenta sobre la dieta Atkins modificada, elección de pacientes, forma de implementación, diversos controles y efectos adversos. A diferencia de la dieta cetogénica clásica, se inicia sin ayuno ni hospitalización, y no hay restricción proteica, calórica o hídrica, por lo que mejora la palatabilidad y, consecuentemente, la tolerabilidad. Conclusiones. La dieta Atkins modificada es un tratamiento útil para pacientes con epilepsia intratable. La publicación de este consenso ofrece la posibilidad de orientar a nuevos centros en su implementación (AU)

Introduction. Epilepsy is a chronic disease that affects 0.5-1% of the population. One third of the patients become refractory to antiepileptic drugs. Among the non-pharmacological treatments available, the modified Atkins diet is an effective treatment used since 2003 as another alternative for children and adults with refractory epilepsy. Development. The Ketogenic Diet National Committee, which depends on the Argentine Society of Pediatric Neurology, elaborated this consensus on the modified Atkins diet, basing itself on a review of the literature and on their clinical experience. This consensus in Spanish explains the different aspects to be taken into account regarding the modified Atkins diet, patient selection, implementation, different controls and adverse effects. Unlike the classic ketogenic diet, the modified Atkins diet is initiated without fasting or hospital stay, nor does it require protein, calorie or fluid restriction, thus improving patient palatability and consequently patient tolerability. Conclusions. The modified Atkins diet is a useful treatment for patients with intractable epilepsy. The publication of this consensus offers the possibility for new centers to get oriented regarding this diet implementation (AU)

Tratamiento de la epilepsia refractaria con dieta de Atkins modificada / Treatment of refractory epilepsy with the modified Atkins diet

La dieta de Atkins modificada (DAM) es una alternativa dietaria terapéutica en el tratamiento de la epilepsia fármaco-resistente. Consiste en una dieta con un aporte de 60% de grasas, 30% de proteínas y 10% de carbohidratos. El objetivo es presentar una serie de 9 pacientes con diagnóstico de epilepsia refractaria de diferentes etiologías, que recibieron tratamiento con DAM en nuestro hospital. En nuestro grupo de 9 pacientes, obtuvimos resultados similares a los publicados por otros autores, con buena adherencia, tolerancia y respuesta. Del total de pacientes, dos lograron una reducción en más del 90% del número de crisis; cuatro, del 50-90%; dos, menos del 50% de control; y solo uno no presentó respuesta a la DAM. Ningún paciente presentó aumento del número de crisis, y fue bien tolerada en todos los casos

The modified Atkins diet (MAD) is an alternative therapeutic diet for the treatment of drug-resistant epilepsy. It consists of a diet with 60% energy from fat, 30% from protein, and 10% from carbohydrates. The objective of this article is to present a series of nine patients diagnosed with refractory epilepsy of different etiologies treated with the MAD at our hospital. In our group of nine patients, results obtained were similar to those published by other authors, with adequate adherence, tolerance and response. Of all patients, two achieved a reduction of more than 90% in the number of seizures; four experienced a reduction of 50-90%; two had a reduction of less than 50% in seizure control; and only one patient did not respond to the MAD. No patient had an increase in the number of seizures, and the diet was well-tolerated in all cases.

Treatment of refractory epilepsy with the modified Atkins diet.

The modified Atkins diet (MAD) is an alternative therapeutic diet for the treatment of drug-resistant epilepsy. It consists of a diet with 60% energy from fat, 30% from protein, and 10% from carbohydrates. The objective of this article is to present a series of nine patients diagnosed with refractory epilepsy of different etiologies treated with the MAD at our hospital. In our group of nine patients, results obtained were similar to those published by other authors, with adequate adherence, tolerance and response. Of all patients, two achieved a reduction of more than 90% in the number of seizures; four experienced a reduction of 50-90%; two had a reduction of less than 50% in seizure control; and only one patient did not respond to the MAD. No patient had an increase in the number of seizures, and the diet was well-tolerated in all cases.

Treatment of refractory epilepsy with the modified Atkins diet. / Treatment of refractory epilepsy with the modified Atkins diet.

The modified Atkins diet (MAD) is an alternative therapeutic diet for the treatment of drug-resistant epilepsy. It consists of a diet with 60

energy from fat, 30

from protein, and 10

from carbohydrates. The objective of this article is to present a series of nine patients diagnosed with refractory epilepsy of different etiologies treated with the MAD at our hospital. In our group of nine patients, results obtained were similar to those published by other authors, with adequate adherence, tolerance and response. Of all patients, two achieved a reduction of more than 90

in the number of seizures; four experienced a reduction of 50-90

; two had a reduction of less than 50

in seizure control; and only one patient did not respond to the MAD. No patient had an increase in the number of seizures, and the diet was well-tolerated in all cases.