Search

Recent evidence supports a role for genetics in autism, but other findings are difficult to reconcile with a purely genetic cause. Pathological changes in the cerebellum in autism are thought to correspond to an event before 30-32 weeks gestation. Our purpose was to determine whether there is an increased incidence of stressors in autism before this time period. Surveys regarding incidence and timing of prenatal stressors were distributed to specialized schools and clinics for autism and Down syndrome, and to mothers of children without neurodevelopmental diagnoses in walk-in clinics. Incidence of stressors during each 4-week block of pregnancy was recorded. Incidence of stressors in the blocks prior to and including the predicted time period (21-32 weeks gestation) in each group of surveys was compared to the other prenatal blocks. A higher incidence of prenatal stressors was found in autism at 21-32 weeks gestation, with a peak at 25-28 weeks. This does support the possibility of prenatal stressors as a potential contributor to autism, with the timing of stressors consistent with the embryological age suggested by neuroanatomical findings seen in the cerebellum in autism. Future prospective studies would be needed to confirm this finding.

Increasing concern about children in a persistent vegetative state (PVS) prompted a survey of members of the Child Neurology Society regarding aspects of the diagnosis and management of this disorder. Major findings of those responding to this survey (26% response rate) were as follows: (1) 93% believed that a diagnosis of PVS can be made in children, but only 16% believed that this applied to infants younger than 2 months and 70% in the 2-month to 2-year group; (2) a period of 3 to 6 months was believed to be the minimum observation period required before a diagnosis of PVS could be made; (3) 86% believed that the age of the patient would affect the duration of time needed to make the diagnosis of PVS; (4) 78% thought a diagnosis of PVS could be made in children with severe congenital brain malformations; (5) 75% believed that neurodiagnostic studies would be of value and supportive of the clinical diagnosis of PVS; (6) members' opinions as to the average life expectancy (in years) for the following age groups after the patients were considered vegetative were: newborn to 2 months, 4.1; 2 months to 2 years, 5.5; 2 to 7 years, 7.3; and more than 7 years, 7.4; (7) 20% believed that infants and children in a PVS experience pain and suffering; and (8) 75% "never" withhold fluid and nutrition from infants and children in a PVS and 28% "always" give medication for pain and suffering.(ABSTRACT TRUNCATED AT 250 WORDS)

The occurrence of the rare but potentially fatal neuroleptic malignant syndrome must be considered by the surgeon treating a patient who develops hyperthermia, mental abnormalities, autonomic instability, and muscle rigidity after exposure to phenothiazines or other neuroleptic drugs. The dopamine agonist bromocriptine appears to be the treatment of choice in adults and seemed to be effective and well tolerated in our patient. Although the syndrome cannot be prevented, recognition is crucial, since effective general and specific therapy is available. Differentiating neuroleptic malignant syndrome from malignant hyperthermia allows early appropriate treatment with bromocriptine.

It is obviously impossible to determine whether corpus callosotomy is an under-or overutilized procedure in children. However, consistent results for the procedure performed on carefully selected patients have been reported from major epilepsy centers. The procedure generally is well tolerated in children, with very few reported adverse effects. The procedure has been used in the most severe of epileptics with favorable results when patients are selected with the goal of ameliorating generalized atonic, myoclonic, tonic, and tonic-clonic seizures. Well-designed prospective studies are certainly needed to better understand the outcomes of callosotomy, but, until these studies are available, children with severe intractable epilepsy should at least have the procedure considered in the management of their condition.

A case of Rasmussen encephalitis with bilateral involvement and onset at age 2 years is reviewed. Rasmussen encephalitis is a rare progressive disease that causes intractable seizures, cognitive decline, and inflammatory changes in the brain. The neurologic involvement is characteristically unilateral. Bilateral involvement in this case was suspected within 5 months of presentation and was confirmed by bilateral frontal lobe biopsies. The severity and progression of the disease in this case was remarkable and resulted in the patient's death by age 5 years, despite numerous attempted therapeutic interventions. Autopsy findings confirmed the diagnosis of Rasmussen encephalitis with bilateral involvement.

Introduction: The long-term outcome of pediatric patients undergoing corpus callosotomy (CC) for palliative control of medically intractable seizures is presented. Methods: During a 27-year period, 43 patients, 20 years of age or younger, underwent CC for seizure palliation and had a minimum of 1 year follow-up. Seizure reduction and stability of that outcome for individual seizure types and for most disabling seizure were reviewed. Results: Overall, 63% of the seizures documented showed a good response. For the most disabling seizure, 56% of the patients had good outcomes. Changes in outcome status occurred within the first 6 months, and outcome was largely maintained after that point. Conclusion: Callosotomy achieves the goal of seizure palliation in more than half of the patients, with stable, good outcomes being maintained in the majority of patients.

The current practice of sectioning the anterior one-half to two-thirds of the corpus callosum in selected patients with intractable seizure disorders has been predicated on the findings that partial section alone is often effective in controlling certain seizure types and that anterior section is better tolerated than complete callosotomy. In those patients in whom an unsatisfactory seizure response to anterior section has been obtained, completion of the section may then be performed. The benefit of such second stage surgery, in terms of seizure frequency reduction by seizure type, is the subject of this study.

The majority of patients with epilepsy in childhood have benign seizure types and eventually outgrow their seizures. Even young patients with seizures that are considered refractory to medication have a good chance of eventually having a remission.1 However, there is a group of children with severe and truly intractable epilepsy who have multiple seizure types that are devastating to their lives. These children with the most severe of epilepsy may in fact be candidates for corpus callosotomy since it is very unlikely that their seizures will improve as they mature. While the majority of patients who have had corpus callosotomy performed to date have been young adults or adolescents, it is appropriate to consider the surgery in patients of a younger age. Callosotomy in young patients may improve not only seizure control, but also behavior and alertness, which allows better functioning in the school and home. This improvement may have several reasons, including fewer clinical seizures, less subclinical interictal epileptiform activity, and the ability to manage the patient on less medication. Also, the younger patient may tolerate the procedure better than the older patient and have less deleterious effects from the procedure (the “disconnection syndrome,” new speech or memory problems). In fact, none of our young patients have had any of these problems persist postoperatively.2

Background Children with chronic health conditions spend most of their day in the school system. The complexity of illness management and disease sequela can alter their school experience. However, little is known about what educators are concerned about if these children are in their classroom. Objective To assess educators' perceptions of the impact of having children with different chronic health conditions in the classroom. Methods Teachers and other school professionals in 23 elementary schools were surveyed about the impact of having a child with each of 6 chronic health conditions—AIDS, asthma, congenital heart disease, diabetes mellitus, epilepsy, and leukemia—in the classroom. They responded to 13 statements about the potential academic impact on the child, impact on peers, personal risk or liability, and additional time or attention demands for the teacher. Total scores were determined for the degree of perceived impact for each issue and chronic health condition and the proportion of teachers with negative perceptions for different issues. Results The mean total scores (2.4 of 5) showed overall a positive perception by school professionals about children with chronic conditions in the classroom. Acquired immunodeficiency syndrome and epilepsy were perceived to have the most impact and asthma the least impact. The 2 educator issues (time or extra attention and personal risk or liability) were of the most concern to educators. Fifty-three percent were concerned about an emergency occurring with the child in the classroom and 27% were concerned about legal liability. Educator concerns of the risk of classroom emergencies or death were disproportionate to the clinical risk of the conditions especially for epilepsy and congenital heart disease. Conclusions Overall school professionals have positive attitudes about children with chronic health conditions in the classroom, but concerns about specific diseases and issues exist. If parents provide most of the disease information, some of the educators' concerns voiced in this study may not be addressed. Health care professionals can help by providing educators with appropriate information about the risk and functional impact of childhood chronic health conditions.

This paper presents electroencephalographic evidence of bilateral independent temporal lobe spiking during an episode of transient global amnesia. The amnesia occurred during a period of hypotension secondary to sinus bradycardia. The amnesia recurred in absence of bradycardia and hypotension. The temporal lobe spiking leads credence to the thought that transient global amnesia occurs as the result of seizure activity.

✓ The case of a 49-year-old man with traumatic complete T-5 paraplegia of 30 years' duration is presented. Over the last 10 years, he developed cervical myelopathy suggestive of syringomyelia. Work-up, including metrizamide myelography and delayed computerized tomography scanning, revealed an atrophic cord without a syrinx, and a complete block to contrast material at T-5. At operation, the cord was found to be tethered at the original injury site, and was released by transection. This resulted in immediate improvement in the somatosensory evoked potentials and marked postoperative clinical improvement. It is postulated that cord tethering can cause delayed myelopathy in patients with traumatic cord injury. Release of the tethered cord should be considered if a posttraumatic syrinx is not found.

A 51-year-old diabetic woman developed bilateral rhinoorbitocerebral phycomycosis. Successful treatment was accomplished by correction of the ketoacidosis, surgical debridement, and amphotericin B therapy. Rhinoorbitocerebral phycomycosis is a fulminant and frequently fatal disease most often seen in debilitated hosts. This survival suggests that aggressive therapy is indicated in patients with extensive fungal involvement.

To the Editor.— The use of Collins' Law 1 instead of five-year survival rates in the assessment of the risk of recurrence of ependymoma has been advocated. 2 The law states that a tumor grows in a patient at a certain rate unique to that patient; thus, after ablation, the tumor will become symptomatic a second time when it reaches a size comparable with that at the time of initial diagnosis. The longest time over which this amount of regrowth can occur is equivalent to the time that would have been available for the first growth of the tumor had it begun at conception (the age of the patient at the time of first diagnosis plus nine months). A symptom-free ratio may also be defined (independent of age) as the ratio of the symptom-free interval to the interval from Left, Ependymoma of patient at age 10 years (hematoxylin-eosin [H-E], x

Drs Solomon and Plum have written a much needed and concise treatise on seizure disorders that is directed to generalists. The authors are both well known in the field of neurology. Dr Plum presently is Professor of Neurology at Cornell University and is serving as editor of the Annals of Neurology , while Dr Solomon is on the faculty of Cornell University and the Director of Electroencephalography at New York Hospital. While Dr Plum's contributions to neurology are immense, he probably is best known to non-neurologists for his classic, The Diagnosis of Stupor and Coma , written with Jerome Posser, MD. This book is extremely well written and easy to read. There is a discussion of the neurophysiology and neurochemistry involved in seizure disorders that is very understandable and that gives the reader the basic information that is necessary to grasp some of the concepts involved in epilepsy. Various seizure types are

This publication is a collection of articles by well-known authors on various subjects in pediatric neurology and neurosurgery. Although not specifically stated, it seems as if the authors were members of a symposium held at the Barrow Neurological Institute of the St Joseph's Hospital and Medical Center, Phoenix, Ariz. The title is somewhat of a misnomer, since the volume certainly is not a comprehensive text, but a series of topics in the two disciplines. The publication suffers because of the many authors involved, and the quality of the articles is quite variable. Most of the articles are well written and are very interesting. However, many of them present a personal point of view and some of them do not have a list of references, which is a serious omission. The chapter entitled "Prenatal factors affecting development in children," by Prensky and Dodson, is an excellent, concise review of the subject