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IntroductionHemispatial 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.MethodsThirty-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.ResultsOnly 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.ConclusionsThese results imply that reorganization to the contralateral hemisphere occurs peri-hemispherectomy, as there are no perilesional areas to assume function.

Epilepsy affects 80 of patients with Sturge–Weber syndrome; the majority of seizures begin before the age of 1. When seizures are intractable to medications and unihemispheric, hemispherectomy is often advised.

This is a prospective cohort study of 237 children (130 males, 107 females) placed on the ketogenic diet for control of intractable epilepsy (mean age at starting diet 3 years 8 months; age range 2 months to 9 years 10 months); average length of follow‐up was 308 days. There were 133 children on the diet at 1 year and 76 at 2 years. Height and weight measurements were converted into age‐ and sex‐appropriate zscores. There was a rapid drop in weight zscores in the first 3 months. After this initial period, the weight zscore remained constant in children who started the diet below the median weight for their age and sex, although zscores continued to decrease in children starting above the median. There was a small decrease in height zscores in the first 6 months (<0.5); however, there were larger changes by 2 years. There was no difference based on sex for either height or weight. The ketogenic diet generally provides sufficient nutrition to maintain growth within normal parameters over a defined period. Very young children grow poorly on the diet and should be followed‐up carefully over long periods of use.

Alternative medical therapies such as the ketogenic diet are often dismissed by the medical community. We used a “parent-centered” approach to make physicians aware of the ketogenic diet, an alternative therapy for difficult-to control seizures in children. Parents became aware of the diet through national TV and film. Written and video information was made available to them and they were encouraged to ask their physicians about the potential usefulness of the ketogenic diet for their child. Between 1994 and 1999 we assessed the success of this approach with three surveys of all U.S. child neurologists. This “parent-centered” approach increased physician knowledge about the diet, but had little effect on its use. Despite evidence of its effectiveness, physicians continued to reserve its use until children had failed most new anticonvulsants. Physicians say they would use it more often if “it worked better” and if they “had a dietitian with more experience.”

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.

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.

The objective of this study was to determine the relationship between β-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 β-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 β-hydroxybutyrate. Blood β-hydroxybutyrate levels statistically correlated with seizure control (P = .003). Children with blood β-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 β-hydroxybutyrate levels exceeded 2 mmol/L. Seizure control correlates with blood β-hydroxybutyrate levels and is more likely when blood β-hydroxybutyrate levels are greater than 4 mmol/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. (J Child Neurol 2000; 15:787-790).

Rational application of basic pharmacologic principles and therapeutic drug monitoring techniques can now result in seizure control for 70 to 80 per cent of children. The characteristics of several important anticonvulsants, including phenobarbital, phenytoin, carbamazepine, clonazepam, and valproic acid, are described.

Sixty percent of malpractice premiums paid by obstetricians go to cover suits for alleged birth-related cerebral palsy (CP). Yet substantially less than half of that money goes to CP victims, and less than 10 percent of children with CP receive any compensation at all from tort suits. This paper proposes a system that would compensate all children born with CP for most handicap-related expenses, in exchange for which the children would be foreclosed from bringing suits alleging birth-related malpractice. Malpractice would be policed by a state board, which would investigate all CP cases. This proposal would be more equitable than current systems. It would also be less expensive, since it would avoid costly litigation and decrease the cost of obstetrical malpractice insurance.

Standard ethical approaches to decision-making which are based on rights, duties, obligations, beneficence, or best interests often seem inadequate or insufficient when applied to the individual infant, as in the case of Baby Jane Doe. A process approach which takes account of moral theory, but which allows tolerance, within limits, for a possible range of decisions, would appear to offer more reasonable decisions. However, any decision must be based on good facts and accurate prognosis. Pending the availability of medical records on Baby Jane Doe, judgment of the decisions made at Stony Brook must be suspended.

There has been a dramatic resurgence of interest in the ketogenic diet during the past several years. For many children with difficult-to-control epilepsy, the diet presents an alternative approach to trying multiple medications. The ketogenic diet's current success rate, when properly executed, greatly exceeds that of the medications which have recently become available. Its side effects, both cognitive and allergic, appear fewer than most available medications. The ketogenic diet is also cheaper than most new anticonvulsants. Even though we now know thatthe diet works, we still do not know howit works. Nor do we know how most anticonvulsants work. The mechanism of action of the ketogenic diet appears to rely on a fundamental change in the brain's metabolism from that of a glucose-based energy substrate to a ketone-based substrate. This change is, in some fashion, critical to the maintenance of seizure threshold. Why should the source of the energy make a difference in seizure threshold?

Interferon induction by administration of pyran copolymer was utilized in three patients in an attempt to treat chronic measles encephalitis (Dawson's encephalitis). Although the course of the disease was not effected by interferon induction, administration of the drug produced hematologic and urinary alterations resembling the hemolytic-uremic syndrome in one patient. Hematologic studies in two additional patients documented the heparin-like nature of pyran copolymer, and the production of a late hypercoagulable state following its administration.

The effect of 6-azauridine, an inhibitor of RNA synthesis, has been shown by 2 experimental methods to inhibit the replication of measles virus within mammalian cells in vitro.In a direct virus titration in which the drug was incorporated in the agar overlay, 6-AZ not only reduced the infectivity of measles virus but also delayed plaque development. Experiments designed to test the effectiveness of 6-azauridine when added to cultures at intervals after infection, showed that this uridine analog was capable of reducing the amount of infectious virus produced by each infected cell, and that the drug is more effective when added early in the infection cycle.

Extract: The excretion of 15N in the urine following the oral administration of compounds labeled with 15N was evaluated in an infant with congenital hyperammonemia while the infant was receiving a low protein diet. When she received either 15N-glycine or 15NH4Cl, the ratio of [15N-NH3] to [15N-urea] in urine was strikingly elevated with respect to [15N-NH3]/[15N-urea] found in studies of other infants receiving high protein diets. However, reduction of the protein in the diet of the control infants resulted in elevation of the ratio of [15N-NH3] to [15N-urea] in urine after the administration of 15N-glycine or 15NH4Cl. No mode of metabolism specific for congenital hyperammonemia was demonstrated in the studies of orally administered 15NH4Cl. In the metabolism of 15N-glycine, the hyperammonemic infant receiving a low protein diet was different from other infants on both high and low protein diets.In the hyperammonemic infant, 15N-urea was much more readily formed from 15N-citrulline than from 15NH4Cl. On the other hand, the addition of unlabeled citrulline to the diet of this infant did not enhance the synthesis of 15N-urea from ingested 15NH4Cl when compared with the addition of unlabeled ornithine-HCl to the diet.Speculation: The data presented lead to the conjecture that inborn errors of ammonia detoxification which result in clinical disease in surviving patients are “partial” defects, and that “complete” defects in ammonia detoxification are so rapidly lethal as to be seldom recognized clinically.

The major complications of lead ingestion are lead encephalopathy and lead neuropathy. encephalopathy is well known to pediatricians and is the most common conseconsequencquencetoxication in chiin childhood.Lead neuropathy, while more common than encephalopathy in adults, is rare in childhood. Oly nine cases off lead neufopathychildren have been reported in detail in the Englislish languageterature. Thirtyo-e other cases have been mentioned. The scarcity of detailed reports of this condition, plus the fact that early recognition of neuneu rropathyrevent the even more serious sequel of encephalopathy, has prompted this report. REPORT OF A CASE A 3½-year-old Neurowith anemia and wepre-ss of two months' duration. Family history was negative foFamily history loginegative forlneuro-isease. Thehematologic disea ment had been normal, -nd previously he hadl, and free of significant disease. He had exhibited pica for almost two years, and had chewed several holes in the plaster of his apartment.He had been well

Porencephaly, a well-recognized pathological entity, has been difficult to recognize clinically. Multiple etiologies and locations of the porencephalic cysts, resulting in varied clinical manifestations, have made clinical recognition difficult.It is the purpose of this paper to present three cases of porencephaly seen at the Columbia-Presbyterian Medical Center during a six-month period, whose manifestations were sufficiently similar to constitute a clinically identifiable syndrome. The porencephaly in these children was secondary to thrombosis of a major cerebral artery.Derived from the Latin word porus, meaning opening, porencephaly was first used by Heschl1 to describe defects in the cerebral mantel communicating with both the ventricular system and the subarachnoid space. Through usage, the term porencephaly now includes single or multiple cystic cavities in the brain which are not neoplastic in origin, may communicate with either the ventricular system or the subarachnoid space, or have no communication.Heschl1 originally described porencephaly

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THE CATABOLIC deamination and transamination of protein results in the formation of ammonia, a noxious substance that must be detoxified and excreted. Retention of ammonia in adults is usually due to advanced liver disease or portocaval shunts and is often associated with encephalopathy. In contrast, ammonia retention in children is usually due to an inherited metabolic defect of the urea cycle. This article describes an abnormality of urea metabolism, presumably due to a deficiency of carbamyl phosphate synthetase. Although the principal clinical manifestations were those of ammonia intoxication, hyperglycinemia and a persistent acidosis, with intermittent ketosis, and hyperlipemia were also noted. REPORT OF A CASE This 5-week-old girl was transferred to Babies Hospital because of unexplained vomiting and intermittent coma. She was the result of the sixth pregnancy of unrelated parents. The first two pregnancies ended in miscarriages, the third in an ectopic pregnancy. The fourth, a girl, died

A VARIETY of neurological signs may be seen in the patient acutely or chronically intoxicated with diphenylhydantoin sodium (Dilantin). These may appear even with conventional dosages, and, if not recognized, may lead to an extensive, but unwarranted, evaluation for a progressive neurological condition. This report describes four children whose clinical picture of a progressive or degenerative neurological disease prompted diagnostic admission. In each instance, the clinical syndrome was demonstrated to be an unfamiliar or unrecognized manifestation of diphenylhydantoin intoxication.It is the purpose of this report to emphasize that diphenylhydantoin toxicity may mimic a degenerative neurologic disease, and may not be readily diagnosed because of the following reasons: (1) Toxicity may occur with conventional or previously tolerated dosages. (2) Coexisting neurological disease may alter the manifestations of toxicity or impede their recognition. (3) The classical signs of toxicity may not be demonstrable, particularly in younger children. REPORT OF CASES Case

DAWSON'S encephalitis is a progressive degenerative disease of the central nervous system secondary to a chronic viral infection. Histologic, electron microscopic, immunologic, and immunofluorescent studies indicate that the etiologic agent in this disease is the rubeola virus. In addition, the rubeola virus has been cultured from brain tissue obtained at cerebral biopsy from several patients. As a chronic progressive infection of the brain, this disease might be susceptible to antiviral chemotherapy. Preliminary trials suggested that 5-bromo-2′-deoxyuridine (BUDR) might alter the course of the disease.1,2 To further evaluate this preliminary data, as well as to acquire detailed information regarding the course of Dawson's encephalitis, a double-blind study was undertaken using BUDR and placebo. MATERIALS AND METHODS Patients were included in the study if all the following characteristics of Dawson's encephalitis were manifest2: (1) intellectual or personality change; (2) myoclonic jerks; (3) periodic bursts of slow wave or synchronous

The ketogenic diet is effective for treating seizures in children with epilepsy. The Atkins diet can also induce a ketotic state, but has fewer protein and caloric restrictions, and has been used safely by millions of people worldwide for weight reduction. Six patients, aged 7 to 52 years, were started on the Atkins diet for the treatment of intractable focal and multifocal epilepsy. Five patients maintained moderate to large ketosis for periods of 6 weeks to 24 months; three patients had seizure reduction and were able to reduce antiepileptic medications. This provides preliminary evidence that the Atkins diet may have a role as therapy for patients with medically resistant epilepsy.

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%. (J Child Neurol1999;14:469-471).

Headlines proclaim, "Brain Damaged Child Awarded $10 Million"! "Obstetricians Flee Practice." "Pregnant Women Cannot Find Doctors." You have seen them too frequently. They are signs of the all-too-familiar malpractice insurance crisis. In light of some new proposed solutions, it is worth reviewing the causes of this crisis and approaches to a solution.There are a variety of factors driving the obstetric malpractice crisis and each has several causes. One factor is our increasingly litigious society that seeks compensation for an injury whether or not that injury was another person's fault. The publicity surrounding the occasional huge multimillion-dollar verdicts and the increasing size of settlements and awards in malpractice cases in general have made the tort system a growth industry for lawyers and have made lawsuits an appealing solution for parents who see an otherwise bleak and expensive future for their impaired child. A second factor is pure misinformation. Because cerebral

• Sodium valproate (n-dipropylacetic acid) is a newly released anticonvulsant drug with a broad spectrum of activity. Twenty-seven children with uncontrolled seizures, predominantly akinetic and myoclonic, were treated with this drug. Encouraging results were noted in those with head-nodding spells, body drops, and myoclonic jerks ("minor motor" seizures).Side effects included occasional gastrointestinal disturbance. No important hepatotoxic complications were noted. Two persons displayed transient neurologic side effects. Of particular interest was the noticeable improvement in mental status noted in 17 of the 27 patients. Some variations in blood level of other anticonvulsant drugs were noted, emphasizing the importance of monitoring these drugs. Valproate sodium provides important improvement in our ability to manage minor motor seizures.(Am J Dis Child 133:274-276, 1979)

Expert witnesses for plaintiff and defense should be able to provide evidence-based literature to support their testimony in depositions and in the courtroom. Specialty societies, such as the American Academy of Neurology, should be willing to review complaints about unsubstantiated testimony and, at times, publicly discipline offending experts.

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.

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.