RESUMEN
We report on inter-rater agreement in assessing the types of seizures exhibited by one hundred mothers ascertained in a study of the teratogenicity of maternal epilepsy and antiepileptic drugs. A summary of each woman's medical record and a one-page report of her responses to questions about her epilepsy were reviewed independently by six neurologists, three in pediatric neurology and three in adult neurology. Agreement was measured by the kappa statistic and log-linear modeling techniques. The adult neurologists agreed with each other 59% of the time, with the agreement higher when all three used information from the patients' records, such as an EEG, rather than when depending on the patients' responses to questions about their epilepsy. The pediatric neurologists agreed with each other 44% of the time and tended to rely more heavily on information in the patients' records, such as an EEG or a prior diagnosis, compared with the adult neurologists.
Asunto(s)
Epilepsia/diagnóstico , Neurología , Adulto , Electroencefalografía , Femenino , Humanos , Modelos Lineales , Pediatría , Médicos , Embarazo , Estudios RetrospectivosRESUMEN
Walking flexibility depends on use of feedback or reactive control to respond to unexpected changes in the environment, and the ability to adapt feedforward or predictive control for sustained alterations. Recent work has demonstrated that cerebellar damage impairs feedforward adaptation, but not feedback control, during human split-belt treadmill walking. In contrast, focal cerebral damage from stroke did not impair either process. This led to the suggestion that cerebellar interactions with the brainstem are more important than those with cerebral structures for feedforward adaptation. Does complete removal of a cerebral hemisphere affect either of these processes? We studied split-belt walking in 10 children and adolescents (age 6-18 years) with hemispherectomy (i.e. surgical removal of one entire cerebral hemisphere) and 10 age- and sex-matched control subjects. Hemispherectomy did not impair reactive feedback control, though feedforward adaptation was impaired in some subjects. Specifically, some showed reduced or absent adaptation of inter-leg timing, whereas adaptation of spatial control was intact. These results suggest that the cerebrum is involved in adaptation of the timing, but not spatial, elements of limb movements.
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Adaptación Fisiológica/fisiología , Retroalimentación/fisiología , Hemisferectomía/rehabilitación , Caminata/fisiología , Adolescente , Encéfalo/fisiopatología , Cerebelo/fisiopatología , Niño , Epilepsia/cirugía , Prueba de Esfuerzo/métodos , Femenino , Humanos , Aprendizaje/fisiología , Masculino , Periodo Posoperatorio , Desempeño Psicomotor/fisiologíaRESUMEN
Tonic and atonic seizures are typically seen in what are often referred to as the catastrophic epilepsies. In simply considering what each of the terms signifies (either the complete loss of tone or a marked increase in tone), they would appear to be at the most diverse ends of the spectrum. They would appear to be as opposite as hot and cold or hard and soft. And yet they are often found to occur in the same patient. This article examines the nature of these seizures and discusses some of the syndromes in which they are seen. Although these seizures are often very difficult to control, some of our medications/therapies have been shown to be effective. Recommendations concerning the efficacy of these therapies and a review of the newer therapies are provided. In addition, the ketogenic diet has been particularly successful in treating these seizures; this is discussed in some detail. Finally, although outcomes for these children are generally less than ideal, many can be helped with a persistent approach that balances seizure control against the side effects of medication.
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Anticonvulsivantes/uso terapéutico , Dieta Cetogénica/métodos , Epilepsia Generalizada/dietoterapia , Epilepsia Generalizada/tratamiento farmacológico , Enfermedad Catastrófica/terapia , Terapia Combinada , Electroencefalografía , Epilepsia Generalizada/terapia , Humanos , Factores de Riesgo , Síndrome , Resultado del Tratamiento , Triglicéridos/uso terapéuticoRESUMEN
Despite over 80 years of use, the ketogenic diet (KD) has never been tested in a blinded manner. Twenty children with intractable Lennox-Gastaut syndrome (LGS) were fasted 36 h and then randomized to receive the classic KD in conjunction with a solution containing either 60 g/day of glucose or saccharin. Parents and physicians were blinded to both the solution composition and level of ketosis. A crossover to the KD with the alternate solution occurred following the sixth day and a repeat fast. A 24-h electroencephalography (EEG) was obtained at baseline and after each arm. After administration of the solution, there was moderate evidence of a reduction in parent-reported seizures between the glucose and saccharin arms, with a median difference of 1.5 seizures per day (p = 0.07). There was no reduction in the number of EEG-identified events, with a median reduction of 7 events per day (p = 0.33). Ketosis was not completely eliminated in the glucose-added arm.
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Dieta Cetogénica , Epilepsias Mioclónicas/dietoterapia , Epilepsia Generalizada/dietoterapia , Ácido 3-Hidroxibutírico/orina , Niño , Preescolar , Estudios Cruzados , Método Doble Ciego , Electroencefalografía , Epilepsias Mioclónicas/orina , Epilepsia Generalizada/orina , Femenino , Solución Hipertónica de Glucosa/administración & dosificación , Humanos , Lactante , Cetonas/orina , Masculino , Sacarina/administración & dosificación , SíndromeRESUMEN
The ketogenic diet (KD) is an established, effective nonpharmacologic treatment for intractable childhood epilepsy. The KD is provided differently throughout the world, with occasionally significant variations in its administration. There exists a need for more standardized protocols and management recommendations for clinical and research use. In December 2006, The Charlie Foundation commissioned a panel comprised of 26 pediatric epileptologists and dietitians from nine countries with particular expertise using the KD. This group was created in order to create a consensus statement regarding the clinical management of the KD. Subsequently endorsed by the Practice Committee of the Child Neurology Society, this resultant manuscript addresses issues such as patient selection, pre-KD counseling and evaluation, specific dietary therapy selection, implementation, supplementation, follow-up management, adverse event monitoring, and eventual KD discontinuation. This paper highlights recommendations based on best evidence, including areas of agreement and controversy, unanswered questions, and future research.
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Dieta Cetogénica , Epilepsia/dietoterapia , Medicina Basada en la Evidencia , Anticonvulsivantes/uso terapéutico , Niño , Terapia Combinada , Contraindicaciones , Dieta Cetogénica/efectos adversos , Suplementos Dietéticos , Resistencia a Medicamentos , Epilepsia/diagnóstico , Humanos , Grupo de Atención al PacienteRESUMEN
INTRODUCTION: Hemispatial neglect has been well established in adults following acute ischemic stroke, but has rarely been investigated in children and young adults following brain injury. It is known that young brains have a tremendous potential for reorganization; however, there is controversy as to whether functions are assumed by the opposite hemisphere, or perilesional areas in the same hemisphere. Patients with intractable epilepsy who undergo hemispherectomy for treatment are missing the entire cortex on one side following surgery. In these patients, only the opposite hemisphere is available to assume function. Therefore, they provide the unique opportunity to determine in what cases the left or right hemisphere can take over the spatial attention functions of the opposite hemisphere following damage. The objective of this study was to determine the incidence and types of hemispatial neglect in children and young adults following both right- and left-sided hemispherectomy; which types of spatial attention functions can be assumed by the opposite hemisphere; and whether factors like their age at time of surgery, handedness, or gender influence recovery. METHODS: Thirty-two children and young adults who had previously undergone hemispherectomy were administered two tests to evaluate for two types of hemispatial neglect: a gap detection test and a line cancellation test. Egocentric neglect was defined as significantly more omissions of targets on the contralesional versus ipsilesional side of the page (by chi square analysis; p<.05). Allocentric neglect was defined as significantly more errors in detecting contralesional versus ipsilesional gaps in circles. RESULTS: Only one of the patients displayed statistically significant hemispatial egocentric neglect on the line cancellation test, and none of the patients displayed statistically significant egocentric or allocentric neglect on the gap detection test. CONCLUSIONS: These results imply that reorganization to the contralateral hemisphere occurs peri-hemispherectomy, as there are no perilesional areas to assume function.
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Adaptación Fisiológica , Epilepsia/cirugía , Hemisferectomía/efectos adversos , Plasticidad Neuronal , Trastornos de la Percepción/etiología , Recuperación de la Función , Adaptación Psicológica , Adolescente , Adulto , Factores de Edad , Niño , Período Crítico Psicológico , Femenino , Lateralidad Funcional , Humanos , Masculino , Adulto JovenRESUMEN
The ideal treatment of infantile spasms is unclear, but many studies advocate hormonal treatment. In the United States, intramuscular ACTH is most widely used, despite the problematic financial cost and side effect profile. Since September 2007, we have replaced ACTH with high-dose oral prednisolone (40-60 mg/day) according to the 2004 United Kingdom Infantile Spasms Study (UKISS). Ten of 15 (67%) infants with new-onset and previously treated infantile spasms became spasm free within 2 weeks; 4 later recurred. More children with an idiopathic etiology for infantile spasms were spasm free than were symptomatic cases (88% vs 43%, P=0.10). Spasm freedom was equivalent to our most recent 15 infants receiving ACTH, with 13 (87%) responding, P=0.16. Oral prednisolone had fewer adverse effects (53% vs 80%, P=0.10) and was less expensive ($200 vs approximately $70,000) than ACTH. We now routinely recommend oral prednisolone to all families of children with infantile spasms.
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Glucocorticoides/administración & dosificación , Prednisolona/administración & dosificación , Espasmos Infantiles/tratamiento farmacológico , Administración Oral , Hormona Adrenocorticotrópica/uso terapéutico , Femenino , Humanos , Lactante , Masculino , Estudios RetrospectivosAsunto(s)
Anticonvulsivantes/uso terapéutico , Epilepsia Tipo Ausencia/tratamiento farmacológico , Etosuximida/uso terapéutico , Triazinas/uso terapéutico , Ácido Valproico/uso terapéutico , Niño , Diagnóstico Diferencial , Epilepsia Tipo Ausencia/diagnóstico , Epilepsia Tónico-Clónica/inducido químicamente , Epilepsia Tónico-Clónica/diagnóstico , Humanos , LamotriginaRESUMEN
The ketogenic diet (KD) typically provides a marginally healthy diet and in recent years has been used for more protracted courses of therapy. This results in concern about the impact of the diet on the general health of the child. Studies have demonstrated poor growth, dyslipidemia, kidney stones, and numerous other problems seen less frequently. Major areas of concern are reviewed as well as some emerging evidence of more beneficial long-term health consequences beyond the expected control of seizures.
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Dieta Cetogénica/efectos adversos , Epilepsia/dietoterapia , Niño , HumanosRESUMEN
PURPOSE: To evaluate cortical auditory function, including speech recognition, in children with benign rolandic epilepsy (BRE). METHODS: Fourteen children, seven patients with BRE and seven matched controls, underwent audiometric and behavioral testing, simultaneous EEG recordings, and auditory-evoked potential recordings with speech and tones. Speech recognition was tested under multiple listening conditions. RESULTS: All participants demonstrated normal speech recognition abilities in quiet, as well as normal peripheral and subcortical auditory function. BRE patients performed significantly worse than controls when speech recognition was tested under adverse listening conditions, including background noise. Five BRE patients who were impaired on two or more tests had centrotemporal spiking on awake EEG. There were no significant group differences in the latency or amplitude of early N100 cortical responses to speech or tones. Conversely, the mismatch negativity, a preattentive index of cortical processing that is elicited passively, was absent or prolonged for speech, but not tones, in BRE patients as compared to controls. DISCUSSION: Children with BRE demonstrated specific speech recognition impairments. Our evoked potential findings indicate that these behavioral impairments reflect dysfunction of nonprimary auditory cortex and cannot be attributed solely to attention difficulties. A possible association between auditory impairments and centrotemporal spiking (>1/min) on awake EEG was identified. The pattern of speech recognition impairments observed is a known risk factor for academic difficulties in school-age children. Our results underscore the importance of comprehensive auditory testing, using behavioral and electrophysiological measures, in children with BRE.
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Corteza Auditiva/fisiopatología , Trastornos de la Percepción Auditiva/fisiopatología , Epilepsia Rolándica/fisiopatología , Percepción del Habla/fisiología , Lóbulo Temporal/fisiopatología , Atención/fisiología , Trastornos de la Percepción Auditiva/diagnóstico , Tronco Encefálico/fisiopatología , Niño , Variación Contingente Negativa/fisiología , Diagnóstico Diferencial , Dominancia Cerebral/fisiología , Electroencefalografía , Epilepsia Rolándica/diagnóstico , Potenciales Evocados Auditivos/fisiología , Potenciales Evocados Auditivos del Tronco Encefálico/fisiología , Femenino , Pruebas Auditivas , Humanos , Masculino , Enmascaramiento Perceptual/fisiología , Tiempo de Reacción/fisiología , Procesamiento de Señales Asistido por Computador , Prueba del Umbral de Recepción del HablaRESUMEN
A cohort study was performed of children started on the ketogenic diet for intractable epilepsy from 2000 to 2005 (n = 195). Children who developed kidney stones were compared with those without in terms of demographics, urine laboratory markers, and intervention with urine alkalinization (potassium citrate). Thirteen children (6.7%) developed kidney stones. The use of oral potassium citrate significantly decreased the prevalence of stones (3.2% vs 10.0%, P = .049) and increased the mean time on the ketogenic diet before a stone was first noted (260 vs 149 patient-months, P = .29). The prevalence of kidney stones did not correlate with younger age or use of carbonic anhydrate inhibitors (eg, topiramate or zonisamide) but trended toward higher correlation with the presence of hypercalciuria (92% vs 71%, P = .08). No child stopped the diet due to stones; in fact, the total diet duration was longer (median 26 vs 12 months, P < .001). Kidney stones continue to occur in approximately 1 in 20 children on the ketogenic diet, and no statistically significant risk factors were identified in this cohort. As oral potassium citrate was preventative, prospective studies using this medication empirically are warranted.
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Grasas de la Dieta/efectos adversos , Cálculos Renales/etiología , Cálculos Renales/prevención & control , Citrato de Potasio/administración & dosificación , Administración Oral , Edad de Inicio , Preescolar , Estudios de Cohortes , Grasas de la Dieta/administración & dosificación , Epilepsia/dietoterapia , Epilepsia/tratamiento farmacológico , Femenino , Humanos , Cálculos Renales/epidemiología , Masculino , Estudios Retrospectivos , Factores de Riesgo , Cálculos Urinarios/tratamiento farmacológico , Cálculos Urinarios/etiología , Cálculos Urinarios/prevención & controlRESUMEN
The ketogenic diet is a valuable therapeutic approach for epilepsy, one in which most clinical experience has been with children. Although the mechanism by which the diet protects against seizures is unknown, there is evidence that it causes effects on intermediary metabolism that influence the dynamics of the major inhibitory and excitatory neurotransmitter systems in brain. The pattern of protection of the ketogenic diet in animal models of seizures is distinct from that of other anticonvulsants, suggesting that it has a unique mechanism of action. During consumption of the ketogenic diet, marked alterations in brain energy metabolism occur, with ketone bodies partly replacing glucose as fuel. Whether these metabolic changes contribute to acute seizure protection is unclear; however, the ketone body acetone has anticonvulsant activity and could play a role in the seizure protection afforded by the diet. In addition to acute seizure protection, the ketogenic diet provides protection against the development of spontaneous recurrent seizures in models of chronic epilepsy, and it has neuroprotective properties in diverse models of neurodegenerative disease.
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Encéfalo/efectos de los fármacos , Dieta Baja en Carbohidratos/métodos , Epilepsia/dietoterapia , Cuerpos Cetónicos/farmacología , Animales , Encéfalo/metabolismo , Niño , Epilepsia/metabolismo , HumanosRESUMEN
The ketogenic diet has traditionally been considered an anticonvulsant therapy of last resort, despite excellent efficacy and limited side effects. We hypothesized that the ketogenic diet would have similar results in patients with new-onset epilepsy. A retrospective study was conducted of patients started on the ketogenic diet since 1994. Thirteen of 460 (2.8%) patients were started on the ketogenic diet as early (zero or one prior anticonvulsant) therapy for seizures. Of those remaining on the diet, 60% (6 of 10) had a > 90% seizure reduction at 6 months and 100% (6 of 6) had a > 90% reduction at 12 months. Patients with infantile spasms were as likely to achieve > 50% seizure reduction at 6 months as patients with other seizure types (75% vs 60%; P = .6). The ketogenic diet can be a valuable therapy before epilepsy becomes intractable. In the 13 patients reported, efficacy without side effects was achieved similarly to that with patients with intractable epilepsy.
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Grasas de la Dieta , Epilepsia/dietoterapia , Edad de Inicio , Niño , Preescolar , Resistencia a Medicamentos , Femenino , Humanos , Lactante , Cetonas , Masculino , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Resultado del TratamientoRESUMEN
Auditory processing of speech and nonspeech sounds was studied prospectively in two hemidecorticectomy patients (ages 10-11 years) with Rasmussen's syndrome. We tested auditory word recognition under four listening conditions: in quiet, in noise, after acoustic filtering, and dichotically. Recognition of environmental sounds and discrimination of tones and digitized syllables were also tested. Presurgical testing confirmed normal processing of speech and nonspeech, for both patients, under all listening conditon. One year after surgery, both patients demonstrated intact recognition of words and environmental sounds in quiet but impaired word recognition in noise. The left hemidecorticectomy patient also demonstrated impaired recognition of low-pass filtered words. These findings suggest that either hemisphere can process speech or nonspeech sounds in quiet, whereas both hemispheres are needed to process speech in background noise. Hemispheric contributions to processing speech in noise appear to differ, with the left hemisphere compensating for loss of phonologic information and the right hemisphere compensating for increased attention demands.
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Percepción Auditiva , Corteza Cerebral/fisiología , Lateralidad Funcional , Hemisferectomía , Estimulación Acústica , Atención , Corteza Cerebral/fisiopatología , Niño , Encefalitis/fisiopatología , Encefalitis/cirugía , Femenino , Humanos , Ruido , Discriminación de la Altura Tonal , Estudios Prospectivos , Percepción del HablaRESUMEN
Seizures during the neonatal period have a broad differential diagnosis, many with a specific treatment and prognosis. In the case reported, a combination of dietary and endocrinologic abnormalities resulted in hypocalcemic seizures, which continued despite aggressive correction of serum ionized calcium levels. Serial electroencephalograms (EEG) performed during the hospitalization were markedly abnormal, and treatment with anticonvulsant drugs was considered given the persistence of seizures despite normalization of serum calcium levels. After 4 days of intravenous calcium administration, the seizure activity resolved, and the patient returned to his normal baseline level of functioning. This case highlights the clinical course of neonatal hypocalcemic seizures, EEG findings in several cases, and possible mechanisms for both hypocalcemic precipitation of seizures and anticonvulsant ineffectiveness.
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Hipocalcemia/complicaciones , Convulsiones/diagnóstico , Convulsiones/etiología , Gluconato de Calcio/sangre , Gluconato de Calcio/uso terapéutico , Electroencefalografía , Humanos , Hipocalcemia/tratamiento farmacológico , Lactante , Inyecciones Intravenosas , MasculinoRESUMEN
One of the most significant trends in therapy has been the renewed popularity of the ketogenic diet. Dr. Vining discusses the history of the diet, its possible mechanisms of action and its clinical efficacy.
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Epilepsia/dietoterapia , Niño , Grasas de la Dieta/uso terapéutico , Ayuno/fisiología , Humanos , Cuerpos Cetónicos/metabolismo , Cetosis/metabolismoRESUMEN
CONTEXT: Little prospective long-term information is available on the effect of a ketogenic diet on plasma lipoproteins in children with difficult-to-control seizures. OBJECTIVE: To determine the effect in children with intractable seizures of a high-fat ketogenic diet on plasma levels of the major apolipoprotein B (apoB)-containing lipoproteins, low-density lipoprotein (LDL) and very LDL (VLDL); and the major apolipoprotein A-I (apoA-I)-containing lipoprotein, high-density lipoprotein (HDL). DESIGN, SETTING, AND PATIENTS: A 6-month prospective cohort study of 141 children (mean [SD] age, 5.2 [3.8] years for 70 boys and 6.1 [4.4] years for 71 girls) with difficult-to-treat seizures who were hospitalized for initiation of a high-fat ketogenic diet and followed up as outpatients. This cohort constituted a subgroup of the 371 patients accepted into the ketogenic diet program between 1994 and 2001. A subset of the cohort was also studied after 12 (n = 59) and 24 (n = 27) months. INTERVENTION: A ketogenic diet consisting of a high ratio of fat to carbohydrate and protein combined (4:1 [n = 102], 3.5:1 [n = 7], or 3:1 [n = 32]). After diet initiation, the calories and ratio were adjusted to maintain ideal body weight for height and maximal urinary ketosis for seizure control. MAIN OUTCOME MEASURES: Differences at baseline and 6-month follow-up for levels of total, VLDL, LDL, HDL, and non-HDL cholesterol; triglycerides; total apoB; and apoA-I. RESULTS: At 6 months, the high-fat ketogenic diet significantly increased the mean plasma levels of total (58 mg/dL [1.50 mmol/L]), LDL (50 mg/dL [1.30 mmol/L]), VLDL (8 mg/dL [0.21 mmol/L]), and non-HDL cholesterol (63 mg/dL [1.63 mmol/L]) (P<.001 vs baseline for each); triglycerides (58 mg/dL [0.66 mmol/L]) (P<.001); and total apoB (49 mg/dL) (P<.001). Mean HDL cholesterol decreased significantly (P<.001), although apoA-I increased (4 mg/dL) (P =.23). Significant but less marked changes persisted in children observed after 12 and 24 months. CONCLUSIONS: A high-fat ketogenic diet produced significant increases in the atherogenic apoB-containing lipoproteins and a decrease in the antiatherogenic HDL cholesterol. Further studies are necessary to determine if such a diet adversely affects endothelial vascular function and promotes inflammation and formation of atherosclerotic lesions.
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Dieta , Lípidos/sangre , Convulsiones/dietoterapia , Adolescente , Apolipoproteínas/sangre , Niño , Preescolar , Grasas de la Dieta , Femenino , Humanos , Hiperlipidemias/etiología , Lactante , Cetosis/orina , Lipoproteínas/sangre , Masculino , Estudios ProspectivosRESUMEN
Clinicians typically breathe a sigh of relief when they make the diagnosis of childhood absence epilepsy. The history is classic-a normal young child with myriad brief periods of staring. The clinic visit is powerful when the phenomena are replicated by having the child hyperventilate. Finally, the EEG is definitive. The ability to demonstrate to a family the abrupt eruption and cessation of spike-wave activity provides a framework for them to understand what is happening to their child. Parents are usually reassured that there are very good therapies, that seizures are typically controlled, and that their child will "outgrow" it. However, data from the multicenter Childhood Absence Epilepsy Study Group are rewriting this narrative.(1-3.)
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Epilepsia Tipo Ausencia/diagnóstico , Niño , Electroencefalografía , HumanosRESUMEN
Aware of parental reports of academic variability, we investigated month-to-month fluctuations in cognitive abilities and EEG status by repeated measures testing in six children with benign epilepsy with central-temporal spikes (BECTS). All showed greater than normal test-retest variability. Daytime EEG abnormalities were also variable. Short-term fluctuations in cognitive function appear common in children with BECTS, potentially impacting academic performance.