MRI and autopsy evidence of early maldevelopment of cerebellar vermis and hemispheres in autism raise the question of how cerebellar maldevelopment contributes to the cognitive and social deficits characteristic of autism. Compared with normal controls, autistic patients and patients with acquired cerebellar lesions were similarly impaired in a task requiring rapid and accurate shifts of attention between auditory and visual stimuli. Neurophysiologic and behavioral evidence rules out motor dysfunction as the cause of this deficit. These findings are consistent with the proposal that in autism cerebellar maldevelopment may contribute to an inability to execute rapid attention shifts, which in turn undermines social and cognitive development, and also with the proposal that the human cerebellum is involved in the coordination of rapid attention shifts in a fashion analogous to its role in the coordination of movement.
Chemotherapy appears to be an effective primary postoperative treatment for many malignant brain tumors in young children. Disease control for one or two years in a large minority of patients permitted a delay in the delivery of radiation and, on the basis of preliminary results, a reduction in neurotoxicity. For patients who had undergone total surgical resection or who had a complete response to chemotherapy, the results are sufficiently encouraging to suggest that radiation therapy may not be needed in this subgroup of children after at least one year of chemotherapy.
Complete gross and microscopic neuropathological examinations of 25 children who died with meningomyelocele, the Arnold-Chiari malformation, and hydrocephalus revealed a wide range and frequency of associated central nervous system malformations. The most remarkable of these anomalies were hypoplasia or aplasia of cranial nerve nuclei (20%), demonstrable obstruction of cerebrospinal fluid flow within the ventricular system (92%), cerebellar dysplasia (72%), a disorder of migration of cortical neurons (92%), fusion of the thalami (16%), agenesis of the corpus callosum (12%), and complete or partial agenesis of the olfactory tract and bulb (8%). The anomalies associated with posterior neural tube closure defects can no longer be considered secondary, but rather must be considered part of a spectrum of malformations caused by an unidentified primary insult to the central nervous system. The frequency and pattern of brain malformations associated with neural tube defects of some children with meningomyelocele suggest that such malformations may seriously affect intellectual outcome.
Cranial asymmetry may be present at birth or may develop during the first few months of life. Over the past several years, pediatricians have seen an increase in the number of children with cranial asymmetry, particularly unilateral flattening of the occiput. This increase likely is attributable to parents following the American Academy of Pediatrics "Back to Sleep" positioning recommendations aimed at decreasing the risk of sudden infant death syndrome. Although associated with some risk of deformational plagiocephaly, healthy young infants should be placed down for sleep on their backs. This practice has been associated with a dramatic decrease in the incidence of sudden infant death syndrome. Pediatricians need to be able to properly diagnose skull deformities, educate parents on methods to proactively decrease the likelihood of the development of occipital flattening, initiate appropriate management, and make referrals when necessary. This report provides guidelines for the prevention, diagnosis, and management of positional skull deformity in an otherwise normal infant without evidence of associated anomalies, syndromes, or spinal disease.
The antibiotic concentration of the fluid from either lateral ventricle was determined 104 times in 37 patients through direct ventricular puncture, external ventricular drainage (EVD), or cerebrospinal fluid shunt sampling. The patients were 1 month to 12 years old. When the patients were receiving maximal intravenous antibiotic therapy alone, the concentrations for the most part were below 5 microgram/ml, whereas patients receiving an antibiotic through direct ventricular puncture, EVD, or a shunt reservoir usually had concentrations over 5 microgram/ml. However, wide variations from patient to patient were found with all forms of treatment despite similar dosages. Clustering of the concentration tended to occur in each individual patient. The authors conclude that, to obtain a high concentration of an antibiotic in the ventricular fluid, one should administer it directly into the ventricle.
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