High prevalence of bihemispheric structural and functional defects in Sturge-Weber syndrome.

Abnormal cerebral venous drainage is associated with hypoxia and glucose deprivation, which can account for progressive neurologic deterioration in Sturge-Weber syndrome. Although developmental delay is common in Sturge-Weber syndrome, bihemispheric calcification is uncommon. Computed tomography (CT) and magnetic resonance imaging (MRI) were used to study the neuroanatomy, while single photon emission computed tomography (SPECT) was used concurrently to evaluate perfusion and glucose metabolism using 99mTc hexamethylpropyleneamine oxime (HMPAO) and [18F] fluorodeoxyglucose (FDG), respectively.

Comparative value of thallium and glucose SPECT imaging in childhood brain tumors.

Thallium-201 chloride (201Tl) single-photon emission computed tomography (SPECT) detects a high percentage of histologically and anatomically diverse pediatric brain tumors. Thallium-201 chloride SPECT and F-18 fluoro-deoxy-glucose (18F-FDG) positron emission tomography (PET) are the most commonly used radionuclide techniques in neuro-oncology. Having developed a methodology to image 18F-FDG with SPECT, the authors performed SPECT scans coupled with magnetic resonance imaging to assess the comparative sensitivity of 201Tl and 18F-FDG in 19 children with brain tumors.

[-Anesthesia and analgesia practice patterns in French obstetrical patients-].

Objective: To assess the rate of epidural analgesia (EA) for parturition and the techniques of anaesthesia for Caesarean section (CS).

Study Design: Retrospective study.

Patients: A series of 84,235 deliveries.

Medulloblastoma: time-dose relationship based on a 30-year review.

Purpose: Time-dose relationships have proven important in many cancer sites. This study evaluates the time factors involved in the successful postoperative radiotherapy of medulloblastoma, based on a 30-year experience in a single institution.

Methods And Materials: Fifty-three patients with medulloblastoma received postoperative craniospinal radiotherapy with curative intent between 1963 and 1993.

Neurobehavioral development in Joubert syndrome.

Research on children with Joubert syndrome has focused on brain structural abnormalities and associated clinical symptoms. The degree of developmental delay has not been objectively reported. We investigated the neurobehavioral development of children with Joubert syndrome through neurobehavioral assessment in the largest sample to date.

Ependymoma: results, prognostic factors and treatment recommendations.

Purpose: To review the University of Florida experience in treating ependymomas, analyze prognostic factors, and provide treatment recommendations.

Methods And Materials: Forty-one patients with ependymoma and no metastases outside the central nervous system received postoperative radiotherapy with curative intent between 1966 and 1989. Ten patients had supratentorial lesions, 22 had infratentorial lesions, and 9 had spinal cord lesions.

Medicine and money: an approach to compensating university physicians.

Academic medical centers have been restructuring faculty compensation plans because decreasing clinical revenues have created budgetary problems. We propose an overall compensation strategy designed to reward high performance of university physicians. The approach incorporates Management by Objectives to improve communication of expectations within the organization and provide college of medicine leadership with much needed mechanisms to align faculty compensation with productivity.

"Joubert syndrome" revisited: key ocular motor signs with magnetic resonance imaging correlation.

Joubert syndrome is characterized by episodic hyperpnea and apnea, developmental delay, hypotonia, truncal ataxia, ophthalmologic abnormalities, and vermian dysgenesis. We studied 15 patients with the diagnosis of Joubert syndrome to (1) more fully define the syndrome's clinical features, and (2) correlate the clinical features with magnetic resonance imaging (MRI) findings. Eight of 15 patients had a history of episodic hyperpnea and apnea.

Correlation between gadolinium-diethylenetriaminepentaacetic acid contrast enhancement and thallium-201 chloride uptake in pediatric brainstem glioma.

We previously showed that thallium-201 (201Tl) chloride is accumulated in over 75% of brain tumors, including brainstem gliomas. The imaging of 201Tl with single photon emission computed tomography (SPECT) may require an abnormal increase in permeability of tumor vessels to allow penetration of the blood-brain barrier. To test this hypothesis, we evaluated the correlation between gadolinium enhancement and the degree of 201Tl uptake on SPECT and the contributions of either gadolinium enhancement or 201Tl uptake to the prognosis in children with brainstem gliomas.

Primary intracranial germ cell tumors: clinicopathologic review of 32 cases.

Primary intracranial germ cell neoplasms are rare tumors and constitute a heterogeneous group. We have reviewed 32 cases, over a 21-year period, from the University of Florida. The cases include 22 germinomas, 6 mixed germ cell tumors, and 4 teratomas.

Gene therapy for neurologic disease: benchtop discoveries to bedside applications. 2. The bedside.

Important advances in basic research have made it possible to examine the safety, toxicity, and efficacy of gene therapy in humans for over 5 years. The development of sophisticated gene delivery systems has resulted in approval by the Recombinant DNA Advisory Committee (RAC) of 125 gene therapy or gene marking studies. One of the primary applications of current retroviral-mediated gene insertion technology has been for malignant brain tumors.

[Cesarean sections in France: impact of organizational factors on different utilization rates].

In this study, we analysed the potential impact of organizational factors to explain the variation of cesarean sections' rates. We used a retrospective sample of 84,372 deliveries and two subsamples of low risk deliveries for cesarean sections. We determined different organisational factors that included: juridical and financial status of maternities, their architecture, the type of on-call for obstetricians, pediatrists and anesthetists, the annual number of deliveries and the level of pediatric staff and equipments of the maternities.

[Analysis of delivery facilities and conditions in 1991 in mothers of infants with birth weights below 1500 grams and/or gestational age under 33 weeks].

Aims: To identify maternities (in terms of level of activity and linkage with pediatric services) where, in 1991, mothers gave birth to infants of gestational age less than 33 weeks and/or with birth weight under 1500 g. To analyse factors linked to the probability of choosing a particular maternity as place of delivery for this population and measure the impact of maternal transferts on the rates of deliveries. To estimate the number of neonate transferts which could be avoided with simple recommendations.

Gene therapy for neurologic disease: benchtop discoveries to bedside applications. 1. The bench.

The overall goal of this review is to provide the pediatric neurologist with a theoretical foundation in gene therapy. Gene therapy became feasible in the early 1970s and the first transfer of a foreign gene into humans was approved by the NIH in 1989. Adenovirus, adeno-associated virus, herpes-simplex virus, retroviruses, and other vectors have been used to efficiently transduce genes into cells in vitro and in vivo.

Assessment of laminin-mediated glioma invasion in vitro and by glioma tumors engrafted within rat spinal cord.

Cell motility within central nervous system (CNS) neuropil may be largely restricted yet infiltration by glioma cells is commonly observed. Glioma cells remodel nervous tissue and may assemble extracellular matrix in order to migrate. We examined the rat C6 glioma cell line for laminin expression and response in vitro and following engraftment into rat spinal cord.

Altered cognitive functioning in children with idiopathic epilepsy receiving valproate monotherapy.

Once a child is diagnosed with epilepsy, a primary concern is whether or not the child's behavior and cognitive abilities will be affected by the disease, by the drug prescribed for seizure control, or both. Direct cognitive effects by the epileptic condition have been described. On the other hand, cognitive effects in epilepsy have been attributed to antiepileptic drug therapy.

[Delivery of women with cicatricial uterus].

Cesarean section rate has risen dramatically, particularly in the U.S., over the past 20 years, with results on maternal and perinatal mortality or morbidity and increase of delivery cost.

Intact binocular function and absent ocular torsion in children with alternating skew on lateral gaze.

Background: A form of skew deviation, called alternating skew on lateral gaze, resembles bilateral superior oblique overaction. Oblique muscle overaction has been recently speculated to result from loss of fusion with subsequent "free-wheeling" of the torsional control mechanisms of the eyes, causing sensory intorsion or extorsion with attendant superior or inferior oblique muscle overaction, respectively. We wanted to investigate whether loss of fusion plays a role in the pathogenesis of alternating skew on lateral gaze.

Effects of gemeprost and mifepristone on the mechanical properties of the cervix prior to first trimester termination of pregnancy.

Objective: To study the effects of oral mifepristone and vaginal gemeprost on the mechanical properties of the cervix prior to first trimester termination of pregnancy by vacuum aspiration.

Design: A comparative study. Each patient served as her own control.

A comparative analysis of fall in haemoglobin following abortions conducted by mifepristone (600 mg) and vacuum aspiration.

We compared post-abortion metrorrhagia in 185 women who used the mifepristone (600 mg) plus sulprostone (250 micrograms) drug combination and in 196 women who underwent vacuum aspiration. The patients were monitored for a 2 week period, with haemoglobin being measured on the day of the abortion and 2 weeks later. The women who had used the drug combination experienced a mean fall of 0.

Value of thallium-201 SPECT imaging in childhood brain tumors.

Thallium-201 chloride single photon emission computed tomography (201Tl SPECT) has been proposed as a diagnostic tool in the assessment of patients with brain tumors. We performed SPECT scans coupled with magnetic resonance imaging (MRI) in children with brain tumors to determine the sensitivity and potential value of SPECT in neuro-oncology. Each patient was injected with 2.

High-dose chemotherapy with marrow reinfusion and hyperfractionated irradiation for children with high-risk brain tumors.

Between November 1990 and March 1993, nine pediatric patients with newly diagnosed brain tumors having a high risk of failure with standard treatment received high-dose thiotepa/cyclophosphamide chemotherapy followed by autologous bone marrow infusion and involved-field hyperfractionated radiation therapy. The presenting diagnoses were brainstem glioma (BSG) [6], parietal mixed high-grade oligodendroglioma-astrocytoma [1], thalamic anaplastic astrocytoma [1], and high-grade parietal glioma [1]. Following chemotherapy there were two partial responses, one minor response, three with stable disease, and one with progressive disease.