1. Downs J, Young D, de Klerk N, Bebbington A, Baikie G, Leonard H. {{Impact of scoliosis surgery on activities of daily living in females with Rett syndrome}}.{ J Pediatr Orthop};2009 (Jun);29(4):369-374.
BACKGROUND: Scoliosis is a common orthopaedic complication of Rett syndrome, and surgery is commonly used to reduce asymmetry in cases with severe scoliosis. METHODS: Data from questionnaires administered to caregivers biennially from 2000 to 2006 were used to describe functional skill levels in subjects with Rett syndrome, and within-subject change in 16 subjects with scoliosis surgery were compared with within-subject change in 186 pairs of data from 86 subjects with conservatively managed scoliosis. Postsurgical assessment was conducted after a mean of 17.8 months. RESULTS: Surgery was associated with improved activities of daily living as measured by the WeeFIM for subjects who were wheelchair bound (P = 0.05). Mobility levels, social interaction, communication skills, and the frequency of daytime napping remained similar for the group as a whole. CONCLUSIONS: Improvements in activities of daily living are likely to represent an increase in the quality of life for subjects and caregivers and were mainly found in subjects who were wheelchair bound, indicating that those who were more severely affected were able to benefit from this intervention. LEVEL OF EVIDENCE: Therapeutic study: level III.
2. Kuwagata M, Ogawa T, Shioda S, Nagata T. {{Observation of fetal brain in a rat valproate-induced autism model: a developmental neurotoxicity study}}. {Int J Dev Neurosci};2009 (Jun);27(4):399-405.
Prenatal exposure to chemicals is well known to induce developmental abnormalities in the central nervous system of children. Developmental neurotoxicity (DNT) tests are important to identify neurotoxic agents and prevent neurodevelopmental disorders. We have investigated DNT, focusing on the fetal brain shortly after chemical exposure. To demonstrate a usefulness of a study focusing on the fetal brain in DNT tests, we assessed the fetal brain in a rat valproate-induced autism model. Rats were treated with sodium valproate (VPA, 800 mg/kg) orally on gestational day (GD) 9 or 11 (VPA9 or VPA11), and the fetal brains were examined on GD16 using immunohistochemistry for serotonin (5-HT), tyrosine hydroxylase (TH), and TuJ1 (neuron specific class III beta-tubulin). Hypoplasia of the cortical plate was induced in both VPA9 and VPA11 groups. Abnormal migration of TH-positive and 5-HT neurons, possibly due to the appearance of an abnormally running nerve tract in the pons, was observed only in the VPA11 group. In addition, when we compared the incidence of these abnormalities between pregnant rats mated in our own animal facility (in-house group), and rats purchased pregnant (supplier group), the supplier group was much more sensitive, especially to the pons abnormality. Shipping stress may affect the reproducibility of VPA-induced DNT. The present results demonstrate that examination of the GD16 fetal brain was useful for detecting and characterizing abnormal development of the brain after VPA exposure. Further discussion was made with reference to the findings in children with autism.
3. Todd J, Mills C, Wilson AD, Plumb MS, Mon-Williams MA. {{Slow Motor Responses to Visual Stimuli of Low Salience in Autism}}. {J Mot Behav};2009 (Mar 24):1-8.
The authors studied 2 tasks that placed differing demands on detecting relevant visual information and generating appropriate gaze shifts in adults and children with and without autism. In Experiment 1, participants fixated a cross and needed to make large gaze shifts, but researchers provided explicit instructions about shifting. Children with autism were indistinguishable from comparison groups in this top-down task. In Experiment 2 (bottom-up), a fixation cross remained or was removed prior to the presentation of a peripheral target of low visual salience. In this gap-effect experiment, children with autism showed lengthened reaction times overall but no specific deficit in overlap trials. The results show evidence of a general deficit in manual responses to visual stimuli of low salience and no evidence of a deficit in top-down attention shifting. Older children with autism appeared able to generate appropriate motor responses, but stimulus-driven visual attention seemed impaired.
