Faculty Bibliography

In 3 children who had undergone cerebral hemispherectomy (hemidecortication) between the ages of 1 year 5 months and 4 years for the alleviation of intractable epilepsy, cerebral glucose utilization was studied serially with positron emission tomography. Three to 7 months after hemispherectomy, glucose utilization in the caudate nuclei on the side of hemispherectomy had decreased to below preoperative values, presumably due to total deprivation of ipsilateral cortical input. One to 2.5 years after surgery, complete restoration of glucose metabolic activity to preoperative levels was seen in 2 patients and partial recovery was seen in 1 patient. These alterations of cerebral glucose utilization are believed to reflect microscopic anatomical reorganizational changes (e.g., collateral sprouting) that have been documented following similar lesions in several animal models. Our findings suggest that positron emission tomography may provide a sensitive measure of developmental brain plasticity in vivo.

We describe a 21-year-old man who developed copper deficiency manifested as a demyelinating neuropathy, chronic intestinal pseudo-obstruction, osteoporosis, testicular failure, retinal degeneration, and cardiomyopathy with a tortuous aorta. His serum copper was low and did not increase despite administration of large doses of intravenous copper sulfate. The ceruloplasmin level as measured by an antibody technique was normal, yet ceruloplasmin (Cp) oxidase activity was very low. The Cp amino acid sequence was normal. This suggests that the copper deficiency was caused by a defect in hepatic processing of copper for incorporation into Cp.

The present study investigated a recently developed benzodiazepine, midazolam, as an intravenous adjuvant to ketamine in infant monkeys undergoing prolonged sedation during positron emission tomography of the brain and heart. Subjects were two rhesus macaque (Macaca mulatta) and ten vervet monkeys (Cercopithecus aethiops sabaeus) ranging in age from 26 to 260 days. Midazolam was an effective intravenous adjuvant to ketamine. This treatment regime resulted in complete immobilization of the animals. Ketamine infusion rates did not vary significantly with age for either species. Sensitivity to midazolam appeared to be age-dependent in vervets because significantly lower dose rates were sufficient to maintain anesthesia in older animals. For rhesus monkeys, midazolam dose rates decreased until approximately 4 months of age, and increased gradually thereafter. © 1993 Wiley-Liss, Inc.

This study examined the presurgical 2-deoxy-2[18F]-fluoro-D-glucose positron emission tomography (PET) patterns of regional cerebral glucose metabolism in the non-resected hemisphere of 13 children who underwent hemispherectomy for early-onset intractable seizures. These patterns were compared with the rate of change in the children's non-verbal communication scores, measured before and after surgery. Irrespective of the side of surgery, the pre-operative glucose metabolism in the non-resected prefrontal cortex correlated significantly with the postoperative rate of change in the children's ability to focus the attention of an adult on an object or event (joint attention). These preliminary findings suggest that pre-operative PET patterns might be associated with certain aspects of the developmental outcome of children undergoing hemispherectomy.