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The ketogenic diet that is used to treat children with severe seizures may increase their risk of developing heart disease by increasing blood cholesterol levels, according to a study of 141 children. A ketogenic diet contains high amounts of fat and low amounts of protein and carbohydrate. This produces substances in the body called ketones.

Objective. The objective of this study was to determine whether the ketogenic diet is safe, well-tolerated, and efficacious in the treatment of infantile spasms. Methods. During a 4-year period, 23 children with infantile spasms, aged 5 months to 2 years, were started on the ketogenic diet; 9 (39%) had symptomatic infantile spasms, and 16 (70%) had hypsarrhythmia. Children had an average prediet exposure to 3.3 anticonvulsants. Two children were enrolled before any medication had been tried. Seizure reduction was analyzed retrospectively, using parent reports and electroencephalograms (EEGs) when available. Results. At 3, 6, 9, and 12 months, 38%, 39%, 53%, and 46%, respectively, of all patients currently on the diet were >90% improved (3 were seizure-free at 12 months); 67%, 72%, 93%, and 100% were >50% improved. Fifty-six percent remained on the diet at 12 months, 46% of whom were >90% improved and 100% were >50%. Fifty percent of those with hypsarrhythmia and follow-up EEGs had EEG improvement. Fifty-seven percent had their medications reduced or discontinued by 12 months. Fifty-seven percent had improvement in development, which was correlated with seizure control. Independent factors that predicted improvement included age younger than 1 year and previous exposure to 3 or fewer anticonvulsants. No child has died, and 7 children had diet-related adverse reactions (nephrolithiasis, gastroesophageal reflux). Discussion. The ketogenic diet is a safe, well-tolerated, and possibly effective potential alternative to other therapies for infantile spasms. Pediatrics 2002;109:780-783; infantile spasms, ketogenic diet, seizures., Infantile spasms are a generalized seizure disorder characterized by clusters of sudden flexor or extensor jerks in infants who are younger than 1 year. (1,2) It is associated with both [...]

Children with one of several severe neurological conditions resulting in repeated seizures may benefit from a surgical procedure involving the removal of half the brain called a hemispherectomy. Life quality measurements were evaluated in 58 children who had had hemispherectomies between 1968 and 1996. Parents and doctors considered all these operations to have been minimally to very successful in improving the patients' overall condition and reducing the number of seizures. Most operations were done on children with Rasmussen's syndrome or birth defects of the brain's physical or vessel structure. Four children died., Purpose. To report the outcomes of the 58 hemispherectomies performed at Johns Hopkins between 1968 and January 1996. Methods. Charts were reviewed of the 58 hemispherectomies performed at Johns Hopkins Medical Institutions by the Pediatric Epilepsy Group during the years 1968 to 1996. Twenty-seven operations were done for Rasmussen's syndrome, 24 operations for cortical dysplasias/hemimegalencephalies, and 7 for Sturge-Weber syndrome or other congenital vascular problems. Seizure control alone did not seem to adequately describe the outcomes of the procedure. Therefore, a score was constructed that included seizure frequency, motor disability, and intellectual handicap. This burden of illness score better described the child's handicap before and after surgery. Results. Perioperative death occurred in 4 out of 58 children. Of the 54 surviving children, 54% (29/54) are seizure-free, 24% (13/54) have nonhandicapping seizures, and 23% (12/54) have residual seizures that interfere to some extent with function. Reduction in seizures was related to the etiology of the unilateral epilepsy. Eighty-nine percent of children with Rasmussen's, 67% of those with dysplasias, and 67% of the vascular group are seizure-free, or have occasional, nonhandicapping seizures. All operations were considered by the parents and the physicians to have been successful in decreasing the burden of illness. In 44 the procedure was very successful, in 7 it was moderately successful, and in 3 it was minimally successful. Success was related to the etiology, and early surgery was preferable. Conclusion. Hemispherectomy can be a valuable procedure for relieving the burden of seizures, the burden of medication, and the general dysfunction in children with severe or progressive unilateral cortical disease. Early hemispherectomy, although increasing the hemiparesis in children with Rasmussen's syndrome, relieves the burden of constant seizures and allows the child to return to a more normal life. In children with dysplasias, early surgery can allow the resumption of more normal development. Pediatrics 1997;100:163-171; hemispherectomy, burden of illness, epilepsy surgery, quality of life., ABBREVIATIONS. CVA, cerebrovascular accident; MDQ motor developmental quotient; IQ intelligence quotient; DQ development quotient; EEG, electroencephalogram. The term hemispherectomy is used throughout the literature for several different neurosurgical procedures designed [...]

Epileptic patients are almost invariably examined with the electroencephalograph (EEG), which often reveals abnormalities in the electrical activity of the brain. It is sometimes possible to identify a single spot, or focus, which serves as the starting point for the seizure activity, which then spreads throughout the brain. Multifocal independent epileptiform discharges (MIED) are indicative of at least three foci affecting both hemispheres. Patients with this form of electrical activity are very often mentally retarded and have signs of diffuse damage to the brain. Surgery is the last recourse for the treatment of severe epilepsy; the neurosurgeon tries to remove the focus at which the seizures start. Therefore, it would appear that epileptic patients with MIED would not be good candidates for surgical treatment, since they have several scattered foci instead of just one. However, the extent to which the EEG patterns correlate with the actual seizures has not been widely studied in patients with MIED. It is becoming popular among neurologists treating epileptic patients to do simultaneous EEG recording and video taping of a patient's seizures. This permits a precise correlation between the seizure and the EEG activity. When this was done for four children with MIED, it was found that the seizure activity in the brain was predominantly the result of a focus or foci in one hemisphere, an observation which was suspected on the basis of the physical movements of the patients during their seizures. Three of the four patients underwent surgery in which extensive portions of their brains were removed. The surgery resulted in improvement in all three patients. In one patient, the seizures were completely controlled, despite the appearance of foci on the EEG which were not surgically removed. The results indicate that surgery may still be considered as a treatment option for severely epileptic children with MIED. (Consumer Summary produced by Reliance Medical Information, Inc.)

Many children who have recurring seizures will outgrow them and suffer no adverse consequences. This is even true for children with febrile seizures, which occur in a child with fever. Parents need to be reassured that the child may not need treatment, especially because many anti-convulsant drugs have severe side effects. A 1999 study showed that a rectal suppository containing the anticonvulsant diazepam was effective in preventing recurring seizures in children and adults.

Administration of carnitine to children with seizures being treated with valproic acid may have no beneficial effect on the nonspecific symptoms associated with valproic acid therapy. Lack of carnitine in the body has been linked with inborn errors of metabolism and muscle disease. Low levels of carnitine caused by chronic valproic acid therapy may produce symptoms such as fatigue, lack of appetite, poor school performance, and irritability. Thirty-seven children receiving either valproic acid or carbamazepine were evaluated by their parents while taking either carnitine or a placebo. Parents rated indicators such as energy, appetite and alertness. The placebo was reported to have the same effect as carnitine. A definite case for carnitine administration to children on anticonvulsant drugs needs to be made before doctors can continue to administer such an expensive drug as carnitine for nonspecific symptoms., Objective. This study was designed to assess the reported improvement in "well-being" perceived by parents when children who are taking anticonvulsant medications are administered carnitine. Methodology. Forty-seven children with seizures who were taking either valproic acid or carbamazepine were enrolled in a placebo-controlled, double-blinded, crossover study of the effects of oral carnitine administration (100 mg/kilo) on their well-being as perceived by their parents. The well-being scores were assessed weekly by phone and in person at the start and end of each 4-week phase. Results. The children's well-being scores improved weekly when either placebo or carnitine were administered. None of the analyses of improved well-being achieved statistical significance. Conclusion. We believe this study documents the necessity for controlled trials when assessing the subjective, beneficial effects of medications. Carnitine is expensive, costing approximately $.30/kilogram of body weight per day ($6 per day for a 20 kilo child). It would not appear warranted to administer carnitine prophylactically to children on anticonvulsant medications for alleviating common, nonspecific symptoms. Because there are no reliable clinical or laboratory tests of symptomatic carnitine deficiency caused by anticonvulsant administration, how to identify children in need of carnitine, and when to administer carnitine therapeutically to children receiving valproate or other anticonvulsants is unclear. Pediatrics 1994;93:893-895; well-being, anticonvulsant medications, carnitine.

To the Editor.-- Cote et al[1] have performed a valuable service in their critical analysis of reported adverse sedation events. Some of the pitfalls inherent in this study are pointed [...]

Purpose: Rasmussen syndrome (RS) is a rare form of epilepsy characterized by progressive destruction of a single hemisphere. To characterize the profile of cortical involvement in RS, we studied the pathological changes in the cerebral cortex of 45 hemispherectomies performed at Johns Hopkins Hospital between 1985 and 2002. Methods: The patterns of pathologic changes and stages of cortical abnormalities were studied by histology and immunocy to chemistry methods. The burden of pathology (BP) was quantified in all brain regions of each of the 45 hemispheres. Results: Our study demonstrated significant heterogeneity in the stages of cortical pathology and the multifocal nature of the disease. These stages varied from early inflammation defined by infiltration of T lymphocytes and neuroglial reactions, to more severe stages with extensive neuronal cell death and cavitation of the cerebral cortex. A greater BP was significantly associated with an early age at onset (p = 0.01) and longer duration of disease (p ≤ 0.001). The BP was similar in all brain regions except the occipital lobe, where the BP was significantly lower (p = 0.032). Conclusions: The multifocal distribution of pathologic changes, as well as the heterogeneity in the stages of cortical damage in each patient, is consistent with an ongoing and progressive immune-mediated process of neuronal damage that involves neuroglial and lymphocytic responses, resembling other autoimmune CNS disorders such as multiple sclerosis. [ABSTRACT FROM AUTHOR]

Purpose: Long-term neuropsychological outcome was studied in 71 patients who underwent hemispherectomy for severe and intractable seizures at The Johns Hopkins Hospital between 1968 and 1997 and who agreed to participate. Seizures were due to cortical dysplasias (n = 27), Rasmussen syndrome (n = 37), or vascular malformations or strokes (n = 7). Both presurgical and follow-up results are available and reported for 53 patients. Methods: Patients and caretakers were interviewed, and patients were administered standard measures of intelligence, receptive and expressive language, visual-motor skills, adaptive/developmental functioning, and behavior. Results: Mean age at surgery was 7.2 years. At follow-up, on average 5.4 years after surgery, 65% are seizure free, 49% are medication free, and, of those responding, none rated quality of life as worse than before surgery. Mean IQ was in the 70s for Rasmussen and vascular patients and in the 30s for cortical dysplasia patients. Language and visual-motor skills were consistent with IQ. For Rasmussen patients only, language was significantly more impaired for left than for right hemispherectomy, both before surgery and at follow-up. Adaptive skills were mildly impaired, with greatest impairment in the physical domain. Cognitive measures typically changed little between surgery and follow-up, with IQ change <15 points for 34 of 53 patients; of the remainder, 11 declined and eight improved. Behavior was free of major problems, but social interactions and activities were limited. Conclusions: The most significant predictor of cognitive skills at follow-up was etiology, with dysplasia patients scoring lowest in intelligence and language but not in visual-motor skills. Regardless of etiology, most patients showed only moderate change in cognitive performance at follow-up. [ABSTRACT FROM AUTHOR]

Studies the effectiveness of the ketogenic diet in preventing seizures. Feasibility in evaluating the ketogenic diet's efficacy in atonic or myoclonic seizures; Manner in which the diet can be manipulated; Achievement or elimination of ketosis.