Differences in Activation and Deactivation in Children with Sickle Cell Disease Compared with Demographically Matched Controls.

BACKGROUND AND PURPOSE: Declines in both functional activation and functional connectivity have been reported in patients with sickle cell disease. In this study, we derived the functional and default mode responses to a word stem paradigm in age-, ethnicity-, and background-matched subjects with sickle cell disease and control groups, with the aim of testing whether both networks were similarly attenuated and whether the changes were related to physiologic parameters that characterize sickle cell disease. MATERIALS AND METHODS: Both the functional and default mode responses were obtained from age- and background-matched controls and the sickle cell population by using a visually presented word stem paradigm on a 3T scanner. RESULTS: We observed an attenuated response to both activation and deactivation in the sickle cell disease group. There were no significant differences in the activation response between the 2 groups for the contrast control > sickle cell disease; however, significant differences were observed in the medial parietal cortex, the auditory cortex, and the angular gyrus for the default mode. For the sickle cell group, a significant correlation between the activation z scores and the physiologic parameters was observed; for the deactivation, the results were not significant but the trend was similar. CONCLUSIONS: The results indicate that the physiologic parameters modulate the activation in the expected fashion, but that the effect was weaker for deactivation. Given that significant differences between the 2 groups were only seen for deactivation, additional factors must modulate the deactivation in sickle cell disease.

Development of user-friendly and interactive data collection system for cerebral palsy.

Cerebral palsy (CP) is a permanent motor disorder that appears in early age and it requires multiple tests to assess the physical and mental capabilities of the patients. Current medical record data collection systems, e.g., EPIC, employed for CP are very general, difficult to navigate, and prone to errors. The data cannot easily be extracted which limits data analysis on this rich source of information. To overcome these limitations, we designed and prototyped a database with a graphical user interface geared towards clinical research specifically in CP. The platform with MySQL and Java framework is reliable, secure, and can be easily integrated with other programming languages for data analysis such as MATLAB. This database with GUI design is a promising tool for data collection and can be applied in many different fields aside from CP to infer useful information out of the vast amount of data being collected.

Mining Association Rules for Neurobehavioral and Motor Disorders in Children Diagnosed with Cerebral Palsy.

Children diagnosed with cerebral palsy (CP) appear to be at high risk for developing neurobehavioral and motor disorders. The most common disorders for these children are impaired visual-perception skills and motor planning. Besides, they often have impaired executive functions, which can contribute to problematic emotional adjustment such as depression. Additionally, literature suggests that the tendency to develop these cognitive impairments and emotional abnormalities in pediatric CP is influenced by age and IQ. Because there are many other medical co-morbidities that can occur with CP (e.g., seizures and shunt placement), prediction of what percentages of patients will incur cognitive impairment and emotional abnormality is a difficult task. The purpose of this study was to investigate the associations between possible factors mentioned above, and neurobehavioral and motor disorders from a clinical database of pediatric subjects diagnosed with CP. The study resulted in 22 rules that can predict negative outcomes. These rules reinforced the growing body of literature supporting a link between CP, executive dysfunction, and subsequent neurobehavioral problems. The antecedents and consequents of some association rules were single factors, while other statistical associations were interactions of factor combinations. Further research is needed to include children's comprehensive treatment and medication history in order to determine additional impacts on their neurobehavioral and motor disorders.

White matter damage in asymptomatic patients with sickle cell anemia: screening with diffusion tensor imaging.

BACKGROUND AND PURPOSE: Ischemic injury to the brain is a common complication of SCA. To better understand the neurologic impact of SCA, TBSS were applied to DTI data to investigate white matter injuries in pediatric patients with SCA. MATERIALS AND METHODS: TBSS comparisons of a range of anisotropy and diffusion measures were carried out between age- and background-matched population groups: patients with SCA with no visible lesions, patients with SCA with mild gliosis, and normal controls. RESULTS: TBSS analysis revealed that both SCA populations exhibited reduced anisotropy and increased diffusivity compared with normal controls in multiple brain regions, including the corpus callosum and centrum semiovale. Furthermore, the results suggest that the severity of SCA is positively correlated with the white matter changes in the corpus callosum. CONCLUSIONS: The study demonstrates that TBSS is a viable technique in detecting subtle white matter damage in patients with SCA whose conventional anatomic MR imaging scans show no, or minimal, abnormalities and has the potential to evaluate the neurologic impact of the treatment of SCA.

A randomized, double-blind, placebo-controlled trial of single-dose intravenous secretin as treatment for children with autism.

OBJECTIVE: To determine whether a single injection of intravenous secretin results in measurable improvements in socialization and/or communication skills in children with autism. STUDY DESIGN: Sixty subjects with autism were randomly selected and assigned to either treatment or placebo group. Subjects in the treatment group received 2.0 clinical units of secretin per kilogram of body weight as a single intravenous dose. Subjects in the placebo group received normal saline solution. Neurodevelopmental and behavioral assessments were performed for all subjects before injection and at 3 and 6 weeks after injection. RESULTS: Assessment of language skills and parents' behavioral assessments revealed no significant differences between the treatment and placebo groups. Raters' assessments of severity of autistic symptoms did not differ for the 2 groups at 6 weeks after injection. A marginally statistically significant improvement in autistic behaviors was seen in the treatment group at 3 weeks after injection (P =.051). CONCLUSIONS: A single dose of intravenous secretin does not appear to have significant effects on either parents' perception of autistic behaviors or language skills at 6 weeks after injection. Transient, marginally significant improvements in autistic behaviors may occur in some children.

Scheduled feeding and 24-hour rhythms of N-acetylserotonin and melatonin in rats.

Male rats, kept under a lighting condition of 14-h light, 10-h dark, were subjected to scheduled feeding regimens. Food was available either in the early light phase or the early dark phase. The 24-h rhythms of serum corticosterone and of N-acetylserotonin (NAS) and melatonin (MT) in the pineal and serum were determined. It was found that whereas serum corticosterone and NAS rhythms responded to the feeding schedules, the rhythms of pineal NAS and of serum and pineal MT remained synchronized with the light-dark cycle. These findings indicated that the pineal was not the major source of circulating NAS. Whereas environmental lighting was the dominant "Zeitgeber" for the NAS rhythms in the pineal and the MT rhythms in the pineal and serum, for serum NAS rhythm, food presentation was the stronger Zeitgeber.

Behavioral effects of 5-hydroxy-N-acetyltryptophan, a putative synthetic precursor of N-acetylserotonin.

1. Adult male rats were injected with either 5-hydroxy-N-acetyltryptophan (5-OHNAT), a synthetic putative precursor of N-acetylserotonin, or 5-hydroxytryptophan (5-HTP), the serotonin precursor, and behavioral effects were observed. Pretreatment consisted of carbidopa and nialamide. 2. The behavioral effects of systemic injections of 5-OHNAT and 5-HTP were qualitatively similar i.e. head weaving, forepaw treading, etc., but the effect of 5-OHNAT was appreciably delayed in onset. 3. 5-OHNAT loading may cause an increase in central nervous system 5-HT via initial deacetylation to 5-HTP. Alternatively, 5-OHNAT may cause a delayed increase in brain NAS and the "serotonin syndrome" may reflect increased conversion of 5-HT to NAS.