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There are situations that occur when customers and servicemen have difficulty lighting pilots on appliances after a natural gas or propane system has been shut off or following an out-of-gas call in a propane system. The inability to light the pilot light is believed to be the result of air getting into the gas system. In some circumstances, customers or servicemen will purge the gas line in order to get the “air” out of the lines. Sometimes this results in flammable gas entering the structure leading to an explosion, flash fire or fire, and subsequently property damage, serious injuries, and sometimes death. As a result of several purging incidents involving explosions, including one at the ConAgra Slim Jim plant in Garner, NC, in June 2009, and at the recommendation of the U.S. Chemical Safety Board, the National Fuel Gas Code Committee of NFPA initiated a Tentative Interim Amendment that changed purging requirements [NFPA 54, National Fuel Gas Code, 2009 Edition]. In the propane industry, it has been claimed that after an out-of-gas situation, air is drawn into the propane tank with moisture, which results in the formation of rust and causes the oxidation of the propane odorant ethyl mercaptan. In this scenario, it is claimed that the propane gas was odorless where the line was purged by the unsuspecting serviceman or homeowner leading to the release of dangerous amounts of gas inside a structure. The question arises as to how air can enter a closed gas system, particularly given the presence of safety valves in appliance gas control valves. These authors hypothesize that air can enter a sealed gas system through some gas control valves when a vacuum is created in empty containers or piping due to ambient diurnal temperature changes and solar radiation. To test this hypothesis, pressures and temperatures associated with empty propane containers and weather conditions were measured for several days. The data was then analyzed to determine the magnitude of the pressure changes occurring inside the gas system. The negative pressure values measured during testing were then applied to the inlet side of a number of appliance gas control valves to determine whether these pressures were sufficient to draw air back into the gas system. This paper includes descriptions of the testing and summaries of the results. Test results were also compared to theoretical calculations of the negative pressures in a closed system.

Since the authors’ 1997 review of the ketogenic diet in Advances in Pediatrics, a remarkable increase has been seen in interest in its use, in reports of its effectiveness, and in studies of how the diet works. Although used primarily for treating seizures in children, the diet has also been shown to be useful in treating intractable seizures in adults, and possibly for treating other nonepileptic conditions, such as brain tumors, Alzheimer’s disease, and diabetes. The ketogenic diet is a high-fat, adequate protein, low-carbohydrate diet designed to produce ketosis through mimicking the metabolic changes of starvation. It is carefully calculated to be nutritionally adequate for each individual, providing 90% of needed calories as fat, a minimum of 1 g/kg of protein, and minimal carbohydrates. The ratio of fats to protein and carbohydrate is classically 4:1. The diet and its cousins—the modified Atkins diet, the medium chain triglyceride (MCT) diet, and the low glycemic index treatment—have provided substantial advances in the treatment of childhood seizures. However, because of myths and misunderstandings about the benefits and side effects of the diet, and because of the lack of trained physicians and dieticians to provide the diet, it is used too infrequently. This report summarizes progress made during the past decade and provides information about both the diet’s documented usefulness in childhood epilepsy and its potential uses in other conditions. A considerably fuller discussion of the classic ketogenic diet and its implementation are provided elsewhere [1–3]. The ketogenic diet only should be undertaken only under the medical supervision of a physician and a dietician knowledgeable in its management. Many myths and misconceptions exist about the ketogenic diet, such as: 1. The ketosis of starvation and of the ketogenic diet is dangerous and similar to the ketoacidosis of diabetes. 2. High-fat diets are unpalatable and difficult to calculate and prepare.

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

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.

The purpose of this blinded, crossover study of the ketogenic diet in children with the Lennox-Gastaut syndrome was to confirm, by the addition of 60 g of glucose per day to negate the ketosis, that the effectiveness of the ketogenic diet was neither the result of a placebo effect nor due to parental expectations and commitment. We found that the additional glucose did not significantly alter the frequency of electroencephalography-assessed events, but did decrease the frequency of parent-reported ``drop'' seizures (P = .07). Fasting had substantial effects on both seizures and electroencephalography-assessed events. The diet remained effective in decreasing seizures of the Lennox-Gastaut syndrome at 12 days, 6 months, and 12 months. In conclusion, the ketogenic diet is effective in decreasing the drop seizures of the Lennox-Gastaut syndrome.

Summary Approximately six hours of biofeedback training was given to eight fecally incontinent children with myelomeningocele in order to establish bowel control. Their ages ranged from five to 15 years. The patients were shown a polygraph tracing of the external anal sphincter while they were being encouraged voluntarily to contract the sphincter when the rectum was distended with progressively larger volumes of air in a balloon. Seven of the eight patients showed normal sensation for rectal distension. Following this training period, five of the children had no incontinent periods, and two of these had discontinued enemas or suppositories. A sixth patient had an 80 per cent reduction in the frequency of incontinence. The remaining two did not benefit. At follow-up between 13 and 24 months later, four children were incontinent once a month or less often; two others were incontinent once per day, a considerable decrease from the pre-training period. The two children who failed to learn were still incontinent at follow-up. These results show that fecally incontinent patients with myelomeningocele can learn to evacuate normally or to reduce soiling after a relatively short period of biofeedback training. RESUME Approximativement six heures dcae;apprentissage en biofeedback ont ete donnees a huit enfants incontinents fecaux, porteurs de myelomeningocele, pour obtenir un controle rectal. Les âges allaient de cinq a 15 ans. Les malades pouvaient observer un trace polygraphique du sphincter anal externe pendant quils etaient encourages volontairement a contracter ce sphincter et que le rectum etait progressivement distendu par le gonflement progressif d'un balloon. Sept enfants sur huit resentirent une sensation normale a la distension rectale. A la suite de cette periode d'apprentissage, cinq des six enfants ne presenteent plus de periode d'incontinence et deux d'entre eux purent abandonner lavements et suppositoires. Un sixieme malade presenta une reduction de 80 pour cent de son incontinence. It n'y eut aucun benefice pour les deux cas restants. Lors d'un controle entre 13 et 24 mois plus tard, quatre enfants etaient incontinents une fois par mois ou moins, deux une fois par jour, ce qui traduisait une diminution considerable par rapport a la periode de pre-apprentissage. Les deux enfants qui n'avaient pu beneficier d'un apprentissage etaient toujours incontinents au moment du controTle. Ces resultats montrent que des malades porteurs de myelomeningocele et incontinents fecaux peuvent acquerir une evacuation rectale normale ou reduire leur incontinence apres une periode relativement courte dcar;apprentissage en biofeedback. ZUSAMMENFASSUNG Acht Kinder mit Stuhlinkontinenz bei Myelomeningocele erhielten etwa sechs Stunden ein Biofeedback Training, durch das eine Darmkontrolle aufgebaut werden sollte. Das Alter der Patienten lag zwischen funf und 15 Jahren. Man forderte die Patienten auf, den Sphincter ani externus willkurlich zu kontrahieren, wahrend das Rectum durch ein zunehmend groβeres Luftvolumen in einem Ballon gedehnt wurde und zeigte ihnen gleichzeitig polygraphische Aufzeichnungen vom Sphincter. Sieben der acht Patienten hatten eine normale Empfindung fur die Dehnung des Rectums. Nach dieser Trainingsperiode hatten funf der Kinder keine Inkontinez mehr und zwei davon konnten Klistiere oder Suppositorien absetzen. Bei einer sechsten Patienten war die Inkontinenzhaufigkeit um 80 Prozent reduziert. Die restlichen zwei hatten keinen Erfolg. Eine Kontrolle nach 13 und 24 Monaten: vier Kinder waren einmal im Monat oder seltener inkontinent; zwei andere waren einmal am Tag inkontinent; insgesamt war das eine betrachtliche Verbesserung zu dem Zustand vor dem Training. Die beiden Kinder, die keinen Lernerfolg hatten, waren auch bei den Kontrollen weiterhin inkontinent. Diese Ergebnisse zeigen, daβ stuhlinkontinente Patienten mit Myelomengocele es eriernen konnen, den Stuhl normal zu entleeren oder das Einkoten zu reduzieren, nachdem sie fur eine relativ kurze Zeit ein Biofeedback Training gemacht haben. RESUMEN Se aplico aproximadamente seis horas de aprendizaje de biofeedback a ocho nibos con incontinencia fecal por mielomeningocele, con el objeto de establecer un control intestinal. Sus edades oscilaban entre cinco y 15 anos. A los pacientes se les mostro un trazado poligrafico del esfinter anal externo, al mismo tiempo que se les incitaba a contraer voluntariamente el esfinter cuando el recto estaba distendido por volumenes de aire progresivamente mas grandes gracias a un baton aereo. Siete de los ocho pacientes mostraron una sensation normal de distension rectal. Siguiendo este periodo de entrenamiento, cinco de los ninos no tuvieron periodos de incontinencia y dos de ellos nee esitaron de una forma discontinua de enemas o supositorios. Un sexto paciente tuvo un 80 por ciento de reduction en la frecuencia de la incontinencia. Los otros dos no obtuvieron ningun beneficio. Vistos posteriormente a los 13 y 24 meses, cuatro ninos eran incontinentes una vez al mes o incluso con menor frecuencia, otros dos eran incontinentes una vez al dia, lo que representaba una disminucion considerable en comparacion con el periodo anterior al del entrenamiento. Los dos ninos que habian fallado en el aprendizaje continuaban todavia incontinentes. Estos resultados muestran que los pacientes con incontinencia fecal con meningocele pueden aprender a evacuar normalmente o a reducir la incontinencia depues de un periodo relativamente corto de entrenamiento por biofeedback.

Summary Purpose: ACTH is currently the standard first-line therapy for new-onset infantile spasms, but it has significant side effects. We hypothesized the ketogenic diet (KD), previously reported as beneficial for intractable infantile spasms, would have similar efficacy, but better tolerability than ACTH when used first-line. Methods: We conducted a retrospective chart review of all infants started on the KD (n = 13) and high-dose ACTH (n = 20) for new-onset infantile spasms at our institution since 1996. Results: Infants were spasm-free in 8 of 13 (62%) infants treated with the KD within 1 month, compared to 18 of 20 (90%) treated initially with ACTH, p = 0.06. When effective, median time to spasm freedom was similar between ACTH and the KD (4.0 vs. 6.5 days, p = 0.18). Those treated with ACTH were more likely to have a normal EEG at 1 month (53% vs. 9%, p = 0.02), however, use of the KD led to EEG normalization within 2–5 months in all eight who became spasm-free. In the five children in whom the KD was unsuccessful, four became spasm-free subsequently with ACTH or topiramate immediately. Side effects (31% vs. 80%, p = 0.006) and relapse rate after initial success (12.5% vs. 33%, p = 0.23) were lower with the KD. Discussion: In this retrospective study, the KD stopped spasms in nearly two-thirds of cases, and had fewer side effects and relapses than ACTH. ACTH normalized the EEG more rapidly, however. Further prospective study of the KD as, with a 2-week time limit if unsuccesful, first-line therapy for infantile spasms is warranted.

Electronic monitoring of the fetal heart rate during labor (EFM), originally designed to assess fetal stress and allow the early detection of the compromised fetus, has instead led to increasing maternal morbidity without decreasing fetal morbidity. The unintended consequences of this technologic advance have led to the creation of a pseudodisease and unwarranted intervention in response to its detection. Is it ethical to introduce a new technology without adequate assessment of its possible consequences? Are we about to repeat this (error resulting from the introduction of EFM?) There is increasing interest in monitoring the function of the newborn brain, to enable the early detection of subclinical seizures. The monitor may also be used for assessing brain function in older children and adults who are comatose or paralyzed and cannot appropriately respond to stimuli. Use of this amplitude-integrated electroencephalography (aEEG) in the newborn for detection of seizures and other brain abnormalities is not dissimilar to the use of electronic fetal heart rate monitoring. Whether seizures or subclinical seizures themselves cause harm to the developing nervous system is unclear. The effectiveness of medications for treatment of seizures in the newborn has not been established. Therefore, the consequences of introducing automated EEG for the detection of subclinical neonatal seizures are likely to be similar to the results of the introduction of EFM: creation of another pseudodisease, followed by unwarranted intervention, and increased legal liability. What are the ethics of continued approval and introduction of unevaluated technology? What is the wisdom of its use? Beware of the unintended consequences.

The ketogenic diet, a high fat, adequate protein, low carbohydrate diet, has, during the past decade, had a resurgence of interest for the treatment of difficult-to-control seizures in children. This review traces its history, reviews its uses and side effects, and discusses possible alternatives and the diet’s possible mechanisms of action. Finally, this review looks toward possible future uses of the ketogenic diet for conditions other than epilepsy.

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 z scores. There was a rapid drop in weight z scores in the first 3 months. After this initial period, the weight z score remained constant in children who started the diet below the median weight for their age and sex, although z scores continued to decrease in children starting above the median. There was a small decrease in height z scores in the first 6 months (

Summary: Purpose: To determine the long-term outcome of children with difficult-to-control seizures who remained on the ketogenic diet for 50% controlled at follow-up than were those managed with medications alone (p < 0.05). A statistically significant difference in long-term outcome was noted between those who responded while on the diet, even if they discontinued it before 1 year, and those who did not (p < 0.05), but no statistical correlation was found between length of time that they had remained on the diet and long-term prognosis. Conclusions: Almost half of the children who discontinued the diet during the first year had a decrease in seizures when assessed 3–6 years later. Twenty-two percent of these had become seizure free without surgery. We were unable to ascertain whether this may have been due to new medications. Those who saw some improvement while on the diet were more likely to have a favorable long-term outcome. Resective surgery, in children who were candidates, or vagal nerve stimulation (VNS) implantation, was more likely to result in significant seizure improvement than was management with medications alone. Whether or not the diet was effective, most families did not regret trying it and would recommend it to others.

Recent years have witnessed an increased interest from pediatric neurologists, neuropediatricians, epileptologists and general neurologists in the use of the ketogenic diet (KD) for the management of refractory epilepsies, particularly in children and adolescents. This article summarizes current knowledge on various issues related to its use, as discussed at a recent international workshop. Aspects discussed in some detail include (i) the putative mechanisms responsible for the diet’s anticonvulsant effects, based on results of biochemical and neurophysiological studies in experimental models; (ii) consensus and controversies on the modalities of initiation of the diet, and different protocols of implementation; (iii) indications and contraindications; (iv) efficacy data, also in relation to seizure type, syndromic form and patients age; (v) adverse effects; (vi) methodological aspects related to assessment of the diet’s clinical effects, and perspectives for future research. Overall, the data reviewed indicate that considerable advances have been made in understanding the modes of action of the diet, its efficacy and tolerability profiles and its potential role in different types of epilepsy. Although clinical studies performed to date have important methodological limitations, including suboptimally characterized patients’ populations and an uncontrolled design, a number of innovative, prospective randomized study protocols have been recently proposed and are being implemented. The results of these will hopefully provide much needed high-quality information to better define the role of the diet in the treatment algorithms in different epilepsy syndromes.

Purpose To report the characteristics of a newly recognized clinical entity in congential aniridia that we have termed aniridic fibrosis syndrome . Design Interventional case series. Methods Retrospective chart review of 155 eyes in 80 patients with congenital aniridia was carried out to identify and characterize eyes that had anterior chamber fibrosis. Histopathologic evaluation was performed in three eyes. Results Seven eyes in six patients were identified to have aniridic fibrosis syndrome. All eyes had undergone previous intraocular anterior segment surgery, some eyes with multiple procedures. Seven eyes had undergone cataract surgery with posterior chamber intraocular lens; six eyes had undergone previous implantation of tube shunt devices, and four eyes had undergone previous penetrating keratoplasty. Clinically, the syndrome was characterized by a progressive retrolenticular and retrocorneal membrane that caused forward displacement of intraocular lenses. Surgical findings indicated that the fibrotic membrane also can involve the ciliary body and anterior retina. Histopathologic evidence from three eyes indicated that the extensive fibrotic tissue originated from the root of the rudimentary iris and entrapped the intraocular lens haptics. Endothelial decompensation that was subsequent to the formation of the aniridic fibrosis syndrome was seen in all eyes. Conclusion Aniridic fibrosis syndrome is characterized by the development of a progressive anterior chamber fibrosis. A possible mechanism that promotes the formation of this fibrotic material may be the proximity or touching of intraocular devices on immature vessels in the rudimentary iris found in aniridia. Patients with aniridia with a history of penetrating keratoplasty, intraocular lenses, and tube shunts should be monitored for aniridic fibrosis syndrome; early surgical intervention is recommended.

Summary: 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.

Summary: Purpose: To determine both the efficacy of and compliance with the ketogenic diet in the adolescent population. Methods: A retrospective study of 45 patients, aged 12–19 years, consecutively enrolled in a ketogenic diet program from 1994 to 2002, was performed. Thirty-seven patients were from The Johns Hopkins Medical Institutions; eight were from The University of Texas at Houston. Charts were reviewed, and patients were contacted by telephone. Results: Six months after diet initiation, 28 (62%) of 45 remained on the ketogenic diet, with six (21%) of 28 having 50–90% seizure reduction, and eight (29%) of 28 having >90%. At 12 months, 20 (44%) of 45 remained on the diet, with seven (35%) of 20 having 50–90% seizure reduction and six (30%) of 20 achieving >90% efficacy. Only 22% discontinued the diet for perceived restrictiveness. The mean diet duration was 1.2 years. Patients with multiple seizure types did best, whereas gender, prior seizure frequency, diet ratio, and age did not influence outcome. Patients dependent on parents for daily care were more likely to remain on the diet at 6 months, but had less efficacy. Weight loss (60%) and menstrual dysfunction (45% of female subjects) were the most commonly reported side effects. Conclusions: The ketogenic diet is as well tolerated and efficacious for adolescents with epilepsy as for the general childhood population.

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.”

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 diet9s efficacy and tolerability. Three to 6 years after diet initiation, all 150 families were sent a survey inquiring about their child9s 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.

We investigated the language capabilities of the isolated right hemisphere in 6 children (age, 7-14 years) after left hemidecorticectomy for treatment of Rasmussen's syndrome. Patients were right-handed before surgery and had at least 5 years of normal language development before the onset of seizures. Language testing included speech sound (phoneme) discrimination, single word and phrasal comprehension, repetition, and naming. Within 4 to 16 days after surgery, patients showed improved phoneme discrimination compared with their performance shortly before surgery. Other language functions remained severely impaired until at least 6 months after surgery. By 1 year after surgery, receptive functions were comparable with, or surpassed, patient presurgery performance. Although word repetition was intact by 1 year after surgery, naming remained impaired, and patient speech was limited largely to production of single words. These results suggest that the right hemisphere is innately capable of supporting multiple aspects of phoneme processing. Recovery of higher level receptive and, to a lesser extent, expressive language functions is attributed to plasticity of the right hemisphere, which appears to persist beyond the proposed critical period for language acquisition and lateralization.

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.

The ethical issues to be considered when assisting a family in making decisions about a child with spina bifida have varied in different geographical locations and over the past decades. Although now, in the United States, most infants are detected in utero and most detected pregnancies are terminated, the process by which such decisions should be made rarely seems to meet the standards of a good decision-making process. The small numbers of infants born with spina bifida require careful decision-making by the physician-parent-healthcare team. These decisions must be made in light of conflicting interpretations of current health care regulations and court rulings. MRDD Research Reviews 1998;4:302–307. © 1998 Wiley-Liss, Inc.

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.

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.

A stereotype of the aftermath of a child’s first afebrile seizure seems to be that the child and the panicked parents are brought to the emergency department by ambulance, an IV in place. Most often the seizure has stopped. Blood work is obtained, a computed tomography (CT) scan is done, and then a history and a physical examination are performed—in that order. A neurologist often is then consulted. This sequence usually is followed by a magnetic resonance imaging (MRI) and an electroencephalogram (EEG) scheduled for the following week, because they are difficult to obtain immediately. The cost of such an evaluation is not trivial (Fig 1), and the benefits are dubious. And yet, such a full-press evaluation seems common. Fig 1. *Costs are those of the Johns Hopkins Emergency Department. Charges will vary with the individual emergency department. Amino acids, metabolic work-up etc are additional, and rarely needed. Physicians staffing emergency departments are often trained in adult care where tumors and vascular events are far more common causes of initial seizures. The evaluation of a child with a new-onset afebrile seizure is often erroneously extrapolated from the adult protocols. Physicians are afraid of being “less than complete,” lest there be subsequent malpractice litigation if everything that could be done is not done. But children are not small adults. The causes of first seizures in children and adolescents, their proper evaluation, their consequences, and their treatment should be different from the adult’s treatment. Seventy percent of seizure disorders start in childhood. Two percent of all emergency department visits to the Boston Children’s Hospital over a 33-month period were for children who had had a seizure. Eighteen percent (500 children) had a first nonfebrile seizure. These 500 children are the subject of the study of the value of emergency neuroimaging in this issue by …

We have been asked to discuss the two sides of 3 question, “Should uncomplicated seizures be ated?” In articulating these two sides each of us metimes postures, sometimes exaggerates, and, at les, is pushed to extremes. But most of the time : try to play the roles of those two spokesmen sitg on your two shoulders, whispering as you try help the patient and the family make their decin-i. Before we begin a discussion about treating-or It treating-seizures, let’s begin with some basic finitions concerning seizures and epilepsy. A seizure is defined as a sudden alteration in ator, sensory, or cognitive function caused by an 3ctrical disturbance in the brain. A classification seizures is shown in Table 1. The different types seizures depend on where in the brain the

Twelve children with Rasmussen's encephalitis underwent hemispherectomy and have been followed for an average of 9.15 years. Our decision-making process leads to early, rather than late operation. Because hemiplegia is inevitable with or without operation, earlier operation may allow such children to resume a more normal life. We postulate that earlier operation may prevent some of the intellectual decline that accompanies the continued seizures and their treatment. In view of the variable pathologic findings and lack of evidence of a viral etiology, we suggest either use of the term "progressive unilateral encephalopathy of childhood" or "Rasmussen's syndrome" in recognition of its heritage.

Moral philosophies have rarely helped as I work with families to make the best decision for their child, for the family and for society. What is the proper role for the physician? I have found over a long career as a pediatric neurologist that “benign” paternalism best fi ts my role.

The ketogenic diet was developed in the 1920s as a treatment for intractable childhood seizures when few antiepileptic drugs (AEDs) were available. There are still children whose seizures are refractory even to modern therapy, but use of the ketogenic diet appears to be waning. At Johns Hopkins, we continue to believe that the diet is very effective and well accepted by patients and families. To reevaluate our opinion of the efficacy and acceptability of this form of therapy in patients cared for in the 1980s with the newer AEDs, we analyzed the records of 58 consecutive patients who had been started on the diet. Before using the diet, 80% of the patients had multiple seizure types and 88% were treated with multiple AEDs; these children were among our most intractable patients. Despite this, seizure control improved in 67% of patients with the ketogenic diet, and actuarial analysis indicated that 75% of these improved patients continued the diet for at least 18 months. Sixty-four percent had AEDs reduced, 36% became more alert, and 23% had improved behavior. The improvement in these patients with intractable seizures and the length of time that families maintained the regimen indicate that the ketogenic diet continues to have a very useful therapeutic role in selected patients and their families.

Seizures, both febrile and nonfebrile, are the most common neurologic problem seen in children. Most seizure problems can, and should, be managed by the pediatrician. This article, along with the following one, represents a dialogue—one we often have with pediatricians about the evaluation and management of seizures in children. A seizure is defined as a paroxysmal electrical discharge of neurons in the brain resulting in alteration of function or behavior. A febrile seizure is defined as a seizure caused by fever. The diagnosis of a febrile seizure is one of exclusion. Before the diagnosis is made definite, it must be established that there was not another cause for the seizure. Factors Important in the Diagnosis of Febrile Seizures AGE Febrile seizures are most common between the ages of 6 mo and 5 y. Beyond this range, seizures are more likely to have another etiology. However, this does not mean that every child outside of this age range needs a brain scan and an extensive initial evaluation. TEMPERATURE The height of the fever at the time of the seizure is helpful to know. Anyone can have a seizure if his or her temperature is sufficiently high. Therefore, in the absence of other demonstrable causes, the higher the fever, the more likely that an initial seizure, occurring in the typical age range, was precipitated by the fever.

Seizures, both febrile and nonfebrile, are the most common neurologic problem seen in children. Most seizure problems can, and should, be managed by the pediatrician. This article, along with the following one, represents a dialogue—one we often have with pediatricians about the evaluation and management of seizures in children. A seizure is defined as a paroxysmal electrical discharge of neurons in the brain resulting in alteration of function or behavior. A febrile seizure is defined as a seizure caused by fever. The diagnosis of a febrile seizure is one of exclusion. Before the diagnosis is made definite, it must be established that there was not another cause for the seizure. Factors Important in the Diagnosis of Febrile Seizures AGE Febrile seizures are most common between the ages of 6 mo and 5 y. Beyond this range, seizures are more likely to have another etiology. However, this does not mean that every child outside of this age range needs a brain scan and an extensive initial evaluation. TEMPERATURE The height of the fever at the time of the seizure is helpful to know. Anyone can have a seizure if his or her temperature is sufficiently high. Therefore, in the absence of other demonstrable causes, the higher the fever, the more likely that an initial seizure, occurring in the typical age range, was precipitated by the fever.

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.

Paralysis seen in children with myelomeningocele has been attributed to congenital myelodysplasia. We suspected that paralysis may be due in part to a spinal cord injury caused by exposure of the neural tube to the amniotic fluid. This hypothesis was tested using a fetal rat model of surgically created dysraphism. Each pup from the experimental group of rats in which the spinal cord was intentionally exposed to the amniotic fluid was born with severe deformity and weakness of the hind limbs and tail. Control fetal rats, subjected to the same procedure without directly exposing the spinal cord to the intrauterine environment, were normal at birth. Histological studies of the exposed spinal cord revealed extensive erosion and necrosis, findings similar to those described in children with myelomeningocele. We therefore propose a “two-hit” hypothesis to explain the paralysis seen in children with myelomeningocele: congenital myelodysplasia complicated by an intrauterine spinal cord injury. Intrauterine protection of the exposed spinal cord might prevent some or all of the paralysis. The possible implications of these findings for the future treatment of myelomeningocele are discussed.

Electrode grids were implanted subdurally in 28 patients with epilepsy. In 16 of the 28 patients, an epileptogenic area was located in the speech-dominant left temporal lobe. Recordings made with the grid revealed that the epileptogenic areas in the patients varied widely in extent: the area was confined within the first 10 mm of the temporal lobe in some patients or it was scattered throughout the entire anterior to posterior 80-mm extend in others. Resection of the epileptogenic area was adjusted accordingly in each case. In 6 of 16 patients who were left-hemisphere-dominant for language, up to 55-80 mm from the tip of the temporal lobe was removed, a measure that exceeds the conventional limit of 50 mm from the tip of the dominant hemisphere. In the remaining 12 of the 28 patients, epileptogenic areas were located in a combination of several lobes. In 7 of these 12 patients, the epileptogenic area encompassed the rolandic area; it was removed without deficit in 4 patients and with expected deficit in 3. Of the latter 3 patients, 1 patient underwent hemispherectomy, and a large portion of the epileptogenic rolandic cortex in the frontal and parietal lobes was removed from the other 2. There were 2 cases of grid-related infection, which cleared with antibiotic treatment; there were no lasting complications of grid implantation in any patient. There was no mortality. Electroencephalographic recording and functional mapping using subdural electrode grids allow a tailored, maximal resection of epileptogenic tissue with minimal injury to critical cortex.

In the preface to the 2006 issue on brain monitoring in the neonate, White and Spitzer1 stated that continuous electroencephalogram monitoring “will substantially enhance our understanding of neonatal neurologic injury and markedly improve outcomes for all hospitalized newborn infants.” However, they caution that “access to information often precedes the ability to use it wisely.” In light of the experience of the obstetrical community with electronic fetal monitoring (EFM),2 widespread use of the new amplitude-integrated electroencephalography (aEEG) to diagnose and manage neonatal seizures should be implemented cautiously. EFM was introduced into widespread clinical practice in the early 1960s in an effort to identify a fetus undergoing stress or distress who might develop hypoxic-ischemic encephalopathy and subsequent cerebral palsy.3,4 At the time of its introduction, there were no controlled studies to show that it was preferable to auscultation. Subsequent studies and meta-analyses have shown that although the cesarean-section rate has increased by 40% since the introduction of EFM, the perinatal morbidity and mortality rates have not changed, nor has the incidence of subsequent cerebral palsy.5–7 However, EFM has resulted in a marked increase in maternal morbidity associated with the cesarean sections. Nelson7 noted that three fourths of children who developed cerebral palsy showed no abnormalities on fetal monitoring, and monitoring … Address correspondence to John M. Freeman, MD, 1026 Rolandvue Rd, Towson, MD 21204. E-mail: jfreeman{at}jhmi.edu

Summary: Purpose: Tuberous sclerosis complex (TSC) is a condition that is frequently associated with intractable, early-onset epilepsy, and often is first seen as infantile spasms. If medications fail and no clear epileptogenic tuber is identified, nonpharmacologic therapies are often attempted. The use of the ketogenic diet specifically for children with TSC and epilepsy has not been previously described. Methods: A chart review was performed of patients with TSC treated with the ketogenic diet over a 5-year period at Johns Hopkins Hospital and Massachusetts General Hospital. Results: Twelve children, ages 8 months to 18 years, were identified. Eleven (92%) children had a >50% reduction in their seizures at 6 months on the diet, and 8 (67%) had a >90% response. Five children had at least a 5-month seizure-free response. Diet duration ranged from 2 months to 5 years (mean, 2 years). Conclusions: In this limited-duration case series of 12 patients, the ketogenic diet was a generally effective therapeutic modality for the intractable epilepsy occasionally seen in children with TSC.

Although the success rates and complications of various treatment options for children with intractable epilepsy have been described, the actual expectations of parents for these treatments are less clear. Since 1998, parents at our institution have written their goals in a letter before starting their children on the ketogenic diet. One hundred consecutive letters were evaluated. The most common first goal was seizure improvement, second was anticonvulsant reduction, and third was cognitive improvement. Ninety percent requested improvement in cognition or alertness. These expectations were either met or exceeded at 6 months in 52–60% of children. Achieving or surpassing parental expectations for cognitive improvement correlated with longer diet duration (P = 0.04), but meeting goals for seizure or anticonvulsant reduction did not. Cognitive improvement (P 90% seizure reduction (P = 0.04) at 6 months positively correlated with longer eventual diet duration. Expectations for cognitive improvement need to be discussed prior to beginning the ketogenic diet.

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. ( J Child Neurol 2005;20:31—34).

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.

Rasmussen's encephalitis, originally thought to be a chronic form of viral encephalitis, is now thought to be an autoimmune disease of the brain and is more properly termed Rasmussen's syndrome. Starting in one area of one side of the brain, the disease appears to gradually and progressively involve that side of the brain causing progressive and intractable focal seizures, a hemiparesis, and expressive aphasia when the left hemisphere is involved. Immune therapy with steroids, immunoglobulins, or plasmaphoresis provide only temporary relief from seizures. Neither antibodies to Glu-R3 nor cortical biopsy are helpful in the diagnosis. Hemispherectomy of one form or another is the only curative therapy, and there is no evidence that one form of hemispherectomy is preferable to another. Immuno-ablative therapy may be a therapy of the future.