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.

Hemispherectomy for intractable unihemispheric epilepsy etiology vs outcome.

BACKGROUND: Surgical removal of one hemisphere has been performed for several decades to treat intractable unihemispheric epilepsy. A prior case series focused on the outcomes after 58 surgeries at Johns Hopkins Hospital in 1997. This series, and an additional 53 cases, were reviewed to bring the outcomes up to date. METHODS: Charts of the 111 patients undergoing hemidecortications at the Pediatric Epilepsy Center from 1975 to 2001 were reviewed and families were contacted. Three children died in the immediate perioperative period and three were lost to follow-up immediately after surgery. Follow-up ranged from 3 months to 22 years. RESULTS: Two children died several years later due to intractable seizures. Overall, 65% are seizure-free, 21% have occasional, non-handicapping seizures, and 14% have troublesome seizures. Eighty percent are on one anticonvulsant or none and 89% are able to walk without assistance. Etiology strongly predicted seizure outcome. Patients with migrational disorders are less likely to be seizure-free than all other etiologies (predominantly Rasmussen and congenital vascular injuries) combined (51% vs 71%, p = 0.05). CONCLUSIONS: Hemidecortication continues to be a beneficial procedure in reducing seizure frequency in cases of unilateral cortical epilepsy. Fewer children with migrational disorders are seizure-free.

The postoperative course and management of 106 hemidecortications.

RATIONALE: The excellent long-term outcome for most children undergoing hemispherectomy is well documented. However, the condition of these children in the immediate postoperative period is poorly described. The purpose of this study was to evaluate the short-term issues surrounding hemispherectomy and their management in a series of patients from our institution. METHODS: 106 hemispherectomies were performed at our institution from 1975 to 2001 (102 hemidecortications). Medical records were retrospectively examined for information regarding immediate postoperative problems and care. RESULTS: Three children died in the immediate perioperative period, while 3 others had significant postoperative morbidity. 82% of these children had postoperative fevers (temperature >38.5 degrees C). Of these children, 62% had lumbar punctures. Ten cases had positive CSF growth, of which 6 cases were felt to have actual meningitis. Patients with CSF growth had a significantly longer prior duration of steroid therapy and higher maximum temperature peaks. CSF pleocytosis and an ill clinical appearance neared significance for prediction of CSF growth. Shunting was performed in 19% of all children and was associated with CSF growth. CONCLUSIONS: Postoperative fevers are common after hemidecortication, but meningitis is not. Children with CSF growth tended to appear more ill and have higher temperature spikes and CSF pleocytosis. Shunting was related to CSF growth.

The ketogenic diet: a 3- to 6-year follow-up of 150 children enrolled prospectively.

OBJECTIVE: To document the long-term outcome of the 83 children with difficult-to-control seizures who were enrolled prospectively in a study of the efficacy of the ketogenic diet and who had remained on the diet for 1 year. METHODS: A total of 150 consecutive children were entered prospectively into a study of the ketogenic diet's efficacy and tolerability. Three to 6 years after diet initiation, all 150 families were sent a survey inquiring about their child's current health status, seizure frequency, and current anticonvulsant medications. They were asked about their experience with the diet and reasons for discontinuation. Several telephone attempts were made to contact those who did not respond to the written questionnaire. Responses were entered in an Access database and analyzed. RESULTS: In 1999, 3 to 6 years after initiating the diet, 107 of 150 families responded to a questionnaire. Thirty-five additional families were interviewed by telephone, 4 were lost to follow-up, and 4 children had died, unrelated to the diet. Of the original 150 patient cohort, 20 (13%) were seizure-free and an additional 21 (14%) had a 90% to 99% decrease in their seizures. Twenty-nine were free of medications, and 28 were on only 1 medication; 15 remained on the diet. There were no known cardiac complications. CONCLUSION: Three to 6 years after initiation, the ketogenic diet had proven to be effective in the control of difficult-to-control seizures in children. The diet often allows decrease or discontinuation of medication. It is more effective than many of the newer anticonvulsants and is well-tolerated when it is effective.

Anaesthesia for caesarean section.

Quality and choice in anaesthesia for caesarean section have significantly improved over the last two decades. During this time, general anaesthesia usage has decreased to the point where, in some centres, it is an occasionally used technique for severe fetal distress. This change in practice may have been responsible for the fall in anaesthetic deaths in pregnant women that has occurred over the same period. The boom in regional anaesthesia has improved the aesthetics of childbirth by caesarean section, women's peri-operative comfort, and post-operative analgesia. It has, however, introduced new problems, such as delays in inducing anaesthesia in emergency situations, post-operative immobility and urinary retention. The increase in anaesthetic choices has led to inconsistencies in practice between individual anaesthetists, and between regions and nations. It is therefore impossible for obstetricians to make assumptions about the impact of anaesthesia on their patients. Where possible, anaesthetic protocols and guidelines should exist in every centre, with obstetricians clearly informed of relevant features. Such an approach will prevent inconsistent advice being given to patients and dangerous mistakes occurring. With every aspect of maternity care, a multidisciplinary team approach is in patients' best interests, and anaesthesia for caesarean section is no exception.

Effects of ketogenic diet on development and behavior: preliminary report of a prospective study.

The ketogenic diet is increasingly used for the management of difficult-to-control seizures in children. Here, we describe the first prospective study of the effects of the diet on development, behavior, and parenting stress. Participants were 65 children (36 males, 29 females) with intractable seizures, ages 18 months to 14 years 6 months, enrolled in a prospective study at the Johns Hopkins Hospital, Baltimore, MD, USA, to study the diet's efficacy. Children were assessed before diet initiation and at 1-year follow-up. At follow-up, 52% (34 of 65) children remained on the diet. Mean seizure frequency decreased from 25 per day before diet initiation to less than two per day 1 year later. At follow-up, mean developmental quotient showed statistically significant improvement (p<0.05), with significant behavioral improvements in attention and social functioning. Parental stress was essentially unchanged. No baseline factor examined predicted diet adherence, and the primary reason for diet discontinuation was insufficient seizure control. These preliminary results support prior anecdotal reports of the beneficial effects of the diet on cognition and behavior.

Energy requirements of spasticity.

Direct measurement of energy expended by spasticity in children with severe spastic quadriparesis is difficult. Insertion of an intrathecal baclofen pump in a 13-year-old boy with severe spasticity and profound mental retardation resulted in an estimated 30 to 40% decrease in his spasticity. As he had been on a carefully calculated ketogenic diet and fed by gastrostomy, his precise caloric intake was known. Decrease in spasticity, on the same caloric intake, led to marked weight gain. Reduction of 100 calories intake resulted in new weight stability. It was possible therefore, to estimate indirectly energy used by his spasticity. This 100 calories, representing 34% of calories above his resting energy requirement, corresponded to an independently estimated 30 to 40% of caloric expenditure of his spasticity. It was concluded that when calculation of calories is critical, energy utilization by spasticity must be taken into consideration.

Levetiracetam psychosis in children with epilepsy.

PURPOSE: Levetiracetam is a new anticonvulsant (AED) with a novel mechanism of action. Although it is generally well tolerated with a good cognitive profile, irritability and hostility have been reported in some adults taking levetiracetam. Observations in children are limited; levetiracetam is not yet approved by the Food and Drug Administration for use in children. METHODS: In four young patients, acute psychosis developed within days to months of initiation of levetiracetam for seizures. RESULTS: A 5-year-old girl began having visual hallucinations of spiders in her room 14 days after starting levetiracetam. A 13-year-old boy began having auditory hallucinations, insomnia, and screaming behavior 3 months after initiation of levetiracetam. A 16-year-old girl became acutely agitated, hyperreligious, and had persecutory delusions within 7 days of starting levetiracetam. A 17-year-old girl had auditory hallucinations telling her to sing and yell after 30 days of taking the drug. All four children had dramatic improvement within days of either discontinuing or decreasing the dose of levetiracetam. The three adolescents had historical findings consistent with mild behavioral problems before initiating levetiracetam, and all four patients had prior cognitive deficits. CONCLUSIONS: Reversible treatment-emergent psychosis associated with levetiracetam therapy was observed in four children and adolescents. Whether rapid initiation or prior neurobehavioral problems predispose to this side effect is not established.

Risk factors for urolithiasis in children on the ketogenic diet.

Kidney stones have been associated with use of the ketogenic diet in children with refractory seizure disorders. We performed a case-control study examining risk factors for the development of stones on the ketogenic diet, and prospectively followed children initiating the ketogenic diet to evaluate the incidence of urolithiasis. Clinical characteristics of 18 children presenting with stones (8 uric acid stones, 6 mixed calcium/uric acid stones, 1 calcium oxalate/phosphate stone, 3 stones not evaluated) were compared with characteristics of non-stone-forming children initiating the ketogenic diet at Johns Hopkins since July 1996. Since July 1996, 112 children initiating the ketogenic diet have been followed for development of stones. Follow-up times on the diet range from 2 months to 2.5 years. Of 112 children, 6 have developed stones (3 uric acid, 3 mixed calcium/uric acid stones) (0.8 children developing stones/ 100 patient-months at risk). Comparisons of children presenting with stones on the ketogenic diet with characteristics of the entire cohort initiating the ketogenic diet suggest younger age at diet initiation and hypercalciuria are risk factors for the development of stones. Prospective evaluation of children initiating the ketogenic diet revealed that almost 40% of patients had elevated fasting urine calcium: creatinine ratios at baseline; this increased to 75% after 6 months on the diet. Median urine pH was 5.5 at diet initiation, and remained at 6.0 thereafter. In a subset of patients tested, urinary citrate excretion fell from a mean of 252 mg/24 h pre diet initiation to 52 mg/24 h while on the diet. Uric acid excretion remained normal. Patients maintained on the ketogenic diet often have evidence of hypercalciuria, acid urine, and low urinary citrate excretion. In conjunction with low fluid intake, these patients are at high risk for both uric acid and calcium stone formation.

Descriptive terms for women attending antenatal clinics: mother knows best?

OBJECTIVE: To determine the noun for 'women who attend antenatal clinics' that is most accepted by the women themselves. DESIGN: Cross sectional study. SETTING: Consultant-led antenatal clinics in Cornwall. POPULATION: All women attending consultant-led antenatal clinics over a two-month period. METHODS: The women were surveyed by written questionnaire. MAIN OUTCOME MEASURES: The first, second and third choices of descriptions offered to women attending antenatal clinics. Secondary outcome measures include the relation of maternal age, gestation, civil status, occupation and obstetric history to the individual's choice of description. RESULTS: Questionnaires were received from 446 women, constituting 13% of the antenatal population of Cornwall. Their median age was 28 years and median gestation 22 weeks; 255 (57%) had one or more children and 289 (65%) were married. The most popular choice of description was 'patient' (39% of first choices made), whereas the most accepted description was 'pregnant woman' (26% of totalled second and third choices). While women who selected 'patient' as first choice were slightly younger (mean 27.5 years) than the remaining women (mean 28.4 years), the choice of 'pregnant woman' was not related to any of the other recorded characteristics of the respondents. Commercial terms that consistently were selected least included 'client', 'consumer' and 'customer'. CONCLUSION: Some professional bodies and government organisations have criticised the use of the term 'patient' to describe antenatal women. In this, the largest study to investigate what the women themselves would choose, 'patient' is the most favoured term.

The ketogenic diet: seizure control correlates better with serum beta-hydroxybutyrate than with urine ketones.

The objective of this study was to determine the relationship between beta-hydroxybutyrate levels and seizure control in children on the ketogenic diet. Seventy-four children on the ketogenic diet presenting for routine follow-up visits had blood levels of beta-hydroxybutyrate correlated with their seizure control. Forty-two children admitted for initiation of the ketogenic diet had urine ketones measured by dipstick and correlated with simultaneous blood levels of beta-hydroxybutyrate. Blood beta-hydroxybutyrate levels statistically correlated with seizure control (P = .003). Children with blood beta-hydroxybutyrate levels greater than 4 mmol/L were significantly more likely to have a decrease in seizure frequency than those with levels less than 4 mmol/L. Urine ketones of 4+ (160 mmol/L) were found on dipstick when blood beta-hydroxybutyrate levels exceeded 2 mmol/L. Seizure control correlates with blood beta-hydroxybutyrate levels and is more likely when blood beta-hydroxybutyrate levels are greater than 4 mmo/L. The traditional measurement of urine ketones by dipsticks in children on the ketogenic diet provides a less than optimal assessment of the degree of blood ketosis. Three to four plus (80-160 mmol/L) urine ketones are necessary, but not necessarily sufficient, to achieve optimal seizure control in children on the ketogenic diet. At present, however, urine ketones are the only readily available inexpensive approach to ketone assessment.

Medication cost reduction in children on the ketogenic diet: data from a prospective study.

In a prospective study of the efficacy of the ketogenic diet in children with severe, refractory epilepsy, data were collected on medication changes over 1 year. Cost reductions in medications were calculated over the first year and estimated for a second year. Fifty-seven percent of the children stayed on the diet for 1 year, and 74% of these children had their number of medications reduced. Forty-eight percent of children who stayed on the diet were on no medications at 12 months follow-up. Daily medication costs were reduced by nearly 70%.

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.

The efficacy of the ketogenic diet-1998: a prospective evaluation of intervention in 150 children.

OBJECTIVE: The ketogenic diet is a high-fat, low-protein, low-carbohydrate diet developed in the 1920s for the treatment of children with difficult to control seizures. Despite advances in both the pharmacotherapy and the surgery of epilepsy, many children continue to have difficult-to-control seizures. This prospective study sought to determine the ketogenic diet's effectiveness and tolerability in children refractory to today's medications. METHODS: One hundred fifty consecutive children, ages 1 to 16 years, virtually all of whom continued to have more than two seizures per week despite adequate therapy with at least two anticonvulsant medications, were prospectively enrolled in this study, treated with the ketogenic diet, and followed for a minimum of 1 year. Seizure frequency was tabulated from patients' daily seizure calendars and seizure reduction calculated as percentage of baseline frequency. Adverse events and reasons for diet discontinuation were recorded. RESULTS: The children (mean age, 5.3 years), averaged 410 seizures per month before the diet, despite an exposure to a mean of 6.2 antiepileptic medications. Three months after diet initiation, 83% of those starting remained on the diet and 34% had >90% decrease in seizures. At 6 months, 71% still remained on the diet and 32% had a >90% decrease in seizures. At 1 year, 55% remained on the diet and 27% had a >90% decrease in seizure frequency. Most of those discontinuing the diet did so because it was either insufficiently effective or too restrictive. Seven percent stopped because of intercurrent illness. CONCLUSIONS: The ketogenic diet should be considered as alternative therapy for children with difficult-to-control seizures. It is more effective than many of the new anticonvulsant medications and is well tolerated by children and families when it is effective.

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.