Rights, respect for dignity and end-of-life care: time for a change in the concept of informed consent.

The current concepts of autonomy, surrogate autonomy and informed consent often lead to futile and expensive care at the ends of life. They may impinge on the dignity of the patient as well as subject society to unwarranted expense. In order to provide affordable healthcare for all, these concepts are in need of modification.

A multicenter study of the efficacy of the ketogenic diet.

OBJECTIVE: To determine the efficacy of the ketogenic diet in multiple centers. DESIGN: A prospective study of the change in frequency of seizures in 51 children with intractable seizures who were treated with the ketogenic diet. SETTING: Patients were enrolled from the clinical practices of 7 sites. The diet was initiated in-hospital and the patients were followed up for at least 6 months. PATIENTS: Fifty-one children, aged 1 to 8 years, with more than 10 seizures per week, whose electroencephalogram showed generalized epileptiform abnormalities or multifocal spikes, and who had failed results when taking at least 2 appropriate anti-epileptic drugs. INTERVENTION: The children were hospitalized, fasted, and a 4:1 ketogenic diet was initiated and maintained. MAIN OUTCOME MEASURES: Frequency of seizures was documented from parental calendars and efficacy was compared with prediet baseline after 3, 6, and 12 months. The children were categorized as free of seizures, greater than 90% reduction, 50% to 90% reduction, or lower than 50% reduction in frequency of seizures. RESULTS: Eighty-eight percent of all children initiating the diet remained on it at 3 months, 69% remained on it at 6 months, and 47% remained on it at 1 year. Three months after initiating the diet, frequency of seizures was decreased to greater than 50% in 54%. At 6 months, 28 (55%) of the 51 initiating the diet had at least a 50% decrease from baseline, and at 1 year, 40% of those starting the diet had a greater than 50% decrease in seizures. Five patients (10%) were free of seizures at 1 year. Age, sex, principal seizure type, and electroencephalogram were not statistically related to outcome. CONCLUSION: The ketogenic diet is effective in substantially decreasing difficult-to-control seizures and can successfully be administered in a wide variety of settings.

The effects of stimulus and subject factors on a face matching task.

An initial experiment revealed that, under very rapid viewing conditions, the usual asymmetry for face recognition occurred only for low-detail facial sketches. Photographs and medium-detail sketches failed to show VF asymmetry. A further experiment revealed that the effect was unlikely to be attributable to discrimination difficulties; this experiment, however, replicated the LVF superiority on low-detail faces only for male subjects. These results are briefly examined in the light of Sergent's theoretical interpretation of hemisphere asymmetry (Sergent, J. Percept. Psychophys. 31, 451-461, 1982). They are also discussed in relation to sex differences in cerebral asymmetry.

Conversion reactions in children as body language: a combined child psychiatry/neurology team approach to the management of functional neurologic disorders in children.

Conversion reactions in children represent a form of "body language," a plea for help in a child who has no alternative method of communicating stress. Although conversion reaction may take many forms, this report focuses on those presenting as neurologic dysfunction. The parallel evaluation and treatment of these symptoms by a child psychiatry/pediatric neurology team has demonstrated success in removing the symptom while simultaneously evaluating the underlying stress. Unnecessary medical tests have been avoided and perpetuating factors have been eliminated. Continued family treatment has emphasized health rather than disease and seems to have prevented recurrence.

Intrapartum asphyxia and cerebral palsy.

Signs of presumed hypoxia/asphyxia of the fetus are not uncommon and can be detected during labor, in the delivery room, and during the early neonatal period. Virtually no single sign or symptom has sufficient correlation to enable prediction of later cerebral palsy with a reasonable degree of medical certainty. To attribute cerebral palsy to prior asphyxia with reasonable certainty, there must be evidence that a substantial hypoxic injury occurred and that a sequence of events ensued which would prove the clinical impact of that hypoxic insult. Few cases of cerebral palsy meet these criteria.

The electroencephalogram in pediatric practice: its use and abuse.

Utilized appropriately, the EEG is a helpful diagnostic tool; however, it is currently much abused and much overutilized. Understanding its limitations and its usefulness may lead to less frequent but more appropriate requests for EEGs. Realization that the EEG alone neither diagnoses nor rules out epilepsy and that it is not crucial in the diagnosis or management of "minimal brain damage" should decrease the utilization and abuse of the procedure.

Benign epilepsy of childhood: a speculation and its ramifications.

Most children with generalized tonic-clonic seizures have a benign developmental disorder of seizure threshold that will be outgrown with or without treatment. Such children may have one or more infrequent seizures before adequate threshold is achieved. A small percentage of children will have frequent generalized seizures (epilepsy) due to brain damage or abnormality. Such patients require vigorous anticonvulsant therapy. However, for the children with "benign childhood epilepsy" treatment may be worse than the disease.

Does carnitine administration improve the symptoms attributed to anticonvulsant medications?: a double-blinded, crossover study.

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, cross-over 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.

Computerized axial tomography in neurologic disorders of children.

Computerized axial tomography offers major advantages over air encephalography and angiography for studies of neurologic disorders in children. The low morbidity and the ease of the procedure permit the accurate diagnosis of intracranial pathology in situations where more invasive procedures would not be undertaken. The techniques of the procedure and its uses and limitations are emphasized in a number of neurologic problems of children.

Computerized axial tomography in chronic seizure disorders of childhood.

Ninety-eight children with chronic seizure disorders were studied by computerized axial tomography (CAT). Structural abnormalities were identified in 30% of these children. The greatest incidence of abnormalities was in children with focal motor seizures (43%) and in those whose EEG demonstrated focal slowing together with focal spikes (38%). The most common abnormality was either focal or generalized atrophy (13%). Only about 2% of the abnormalities discovered by CAT were potentially of therapeutic significance. However, the demonstration of a static process, or the finding of a normal CAT, may be quite helpful to the family and physician in certain children with chronic seizure disorders.

Neonatal seizures. I. Correlation of prenatal and perinatal events with outcomes.

A review of 277 newborns with neonatal seizures enrolled in the Collaborative Perinatal Project revealed a mortality of 34.8%. Of the 181 survivors, most followed up to age 7 years, 70% were normal. Thus, despite the fact that seizures are a major indicator of perinatal asphyxia and a predictor of subsequent neurologic deficit, most infants with neonatal seizures who survived did well. Thirteen percent had cerebral palsy, 19% had an IQ less than 70, and 20% had epilepsy. Thirteen percent of survivors had a combination of mental retardation, cerebral palsy, or epilepsy. A low Apgar score, the need for resuscitation after 5 minutes of age, low birth weight, and the early onset of seizures or prolonged seizures correlated with adverse outcome.

Neonatal seizures. II. A multivariate analysis of factors associated with outcome.

A multivariate analysis of perinatal events occurring in infants with neonatal seizures who were enrolled in the National Collaborative Perinatal Project allowed prediction of outcome. This prediction of death or of mental retardation, cerebral palsy, or epilepsy was empirically confirmed 64% to 83% of the time. In an infant with neonatal seizures, a five-minute Apgar score of less than 7, the need for resuscitation after 5 minutes of age, the onset time of the seizures, and a seizure lasting more than 30 minutes are the best early predictors of which infants will die or will have significant neurologic sequelae. It is hypothesized that neonatal seizures may be a better indicator of the severity or duration of intrauterine asphyxia than the Apgar score. In the neonate with seizures, the use of the formula may allow identification of infants at high risk for adverse outcomes.

A new, treatable source of recurrent meningitis: basioccipital meningocele.

A 19-month-old boy suffered eight episodes of bacterial meningitis. During the ninth episode a meningocele of the basioccipital clivus communicating with the nasopharynx was discovered. Identification of the organism causing the episodes of meningitis was not helpful in pointing to the site of this congenital anatomic defect. Surgical closure of the defect has prevented further recurrences.

Long-term outcome of children with severe head trauma and prolonged coma.

Forty-six children with significant head trauma who remained in coma more than 24 hours were the subjects of this long-term outcome study. Twelve (38%) died. The average length of coma in the 34 survivors was 15.5 days. Follow-up ranged from 9 months to 4 years (mean 21 months). Twenty-nine percent of the survivors were normal at follow-up. An additional 53% had mild cognitive or behavioral problems, but 61% of these had evidence of similar problems prior to the injury. Nine percent of survivors had motor residua but normal intellect. Nine percent had severe intellectual and motor problems. Children less than 2 years of age had a worse outcome. Despite severe injury and prolonged coma after head trauma, most children do well. Intensive medical and surgical care increases the rate of survival and does not result in a large number of severely disabled survivors.

Why would you remove half a brain? The outcome of 58 children after hemispherectomy-the Johns Hopkins experience: 1968 to 1996.

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.

Psychologic and behavioral effects of antiepileptic drugs in children: a double-blind comparison between phenobarbital and valproic acid.

Traditional clinical monitoring of children with epilepsy does not appear to be sufficiently sensitive to cognitive functioning and behavioral problems. Although subtle, these changes may alter a child's ability to perform well in school and in society. Physicians must prevent seizures without producing intolerable side effects, and ways of more appropriately assessing these side effects must be developed. In this double-blind, counter-balanced, crossover study of 21 children, the effects of phenobarbital and valproic acid on cognitive functioning and behavior were measured. There was no difference in seizure control between the drugs, and each medication was maintained in the therapeutic range for 6 months (mean phenobarbital level, 21.2 micrograms/mL; mean valproic acid level, 94.1 micrograms/mL). Children were treated with each drug for 6 months. Differences between the drugs were seen on measurements of cognitive function and behavior. On four tests of neuropsychologic function, children performed significantly less well while receiving phenobarbital (P less than .01). There was no evidence that the patients were sedated or less able to perform continuous performance tasks while receiving phenobarbital. Parental assessment of behavior indicated significantly worse behavior with the phenobarbital regimen for three items (P less than .01) and children were measurably more "hyperactive" (P less than .05). Routine clinical assessment of the patients did not reveal differences between the drugs with respect to routine laboratory measurements or side effects as assessed by history or physical examination. Although children may appear to tolerate a medication without clinically apparent problems, subtle but significant changes in intellectual function and behavior may be occurring. Additional, more sensitive, methods of monitoring patients while receiving these drugs is necessary.

Seizures decrease rapidly after fasting: preliminary studies of the ketogenic diet.

OBJECTIVES: To evaluate the change in atonic or myoclonic seizures associated with the Lénnox-Gastaut syndrome during the initiation of the ketogenic diet, and to describe the development of a blinded crossover study of the efficacy of the ketogenic diet. DESIGN: A before-after trial. SETTING: The Johns Hopkins Hospital, Baltimore, Md. PATIENTS: Change in clinical seizure frequency was examined in 17 consecutively treated patients with atonic or myoclonic seizures. In a few patients, a 24-hour ambulatory electroencephalogram was obtained before and after diet initiation. We demonstrated the ability to manipulate the ketosis induced by fasting with the addition of glucose (dextrose) in 1 patient. INTERVENTIONS: Children fasted for 36 hours, and the diet was gradually introduced over 3 days. Parents were instructed to keep a baseline seizure frequency calendar for the month before the initiation of the diet. These calendars continued to be maintained as the diet was initiated. MAIN OUTCOME MEASURE: Seizure decrease from baseline. RESULTS: The atonic or myoclonic seizures decreased in these children by more than 50% immediately. Using a 24-hour ambulatory electroencephalogram, we documented that the seizures reported by a parent represent only a fraction of the electroclinical events; the technique could be used to measure the profound decrease in electrically documented seizures. Ketosis was eliminated with glucose, 60 g/d. CONCLUSIONS: It is feasible to evaluate the ketogenic diet's efficacy in atonic or myoclonic seizures in a blinded, crossover study. The diet can be manipulated on a short-term basis in a blinded manner, and ketosis can be achieved or eliminated.

Carbamazepine levels in pregnancy and lactation.

An epileptic patient whose seizures were controlled with carbamazepine and primidone was followed throughout pregnancy and lactation. Blood levels of primidone decreased during pregnancy and rose postpartum requiring dosage adjustments. Pharmacologically insignificant amounts of the drug were detected in breast milk. A review of the literature revealed 94 infants exposed to carbamazepine in utero and no evidence to date that this drug carries a teratogenic risk.

A clinical index for predicting visual acuity after cataract surgery.

We developed a clinical index for predicting postoperative visual acuity of cataract patients and cross-validated it using data from 182 patients aged 70 years and older. The index consisted of four statistically combined indicators: age, preoperative visual acuity, frequency of reading, and comorbidity. Validation of the index included comparisons to two standard technical instruments for measurement of retinal visual acuity. For the clinical index, 72% of predictions were accurate within one Snellen line of postoperative visual acuity compared to 37% using a laser interferometer and 33% using a potential acuity meter. Testing of the clinical index's external validity using data from 111 patients in a different ophthalmology clinic disclosed 61% of predictions accurate within one Snellen line.

Seizures in adolescents.

The management of a patient with seizures involves proper classification, etiologic evaluation, and administration of appropriate therapy. With proper evaluation and management, control of seizures can be expected in the majority of patients. The physician must realize that an epileptic adolescent has intrinsic anxieties and faces social, educational, and vocational restrictions. Failure to deal with these psychosocial problems may result in a greater handicap to the patient than the seizures themselves.