1. Ballinger EC, Cordeiro L, Chavez AD, Hagerman RJ, Hessl D. {{Emotion Potentiated Startle in Fragile X Syndrome}}. {J Autism Dev Disord};2014 (May 11)
Social avoidance and anxiety are prevalent in fragile X syndrome (FXS) and are potentially mediated by the amygdala, a brain region critical for social behavior. Unfortunately, functional brain resonance imaging investigation of the amygdala in FXS is limited by the difficulties experienced by intellectually impaired and anxious participants. We investigated the relationship between social avoidance and emotion-potentiated startle, a probe of amygdala activation, in children and adolescents with FXS, developmental disability without FXS (DD), and typical development. Individuals with FXS or DD demonstrated significantly reduced potentiation to fearful faces than a typically developing control group (p < .05). However, among individuals with FXS, social avoidance correlated positively with fearful-face potentiation (p < .05). This suggests that general intellectual disability blunts amygdalar response, but differential amygdala responsiveness to social stimuli contributes to phenotypic variability among individuals with FXS.
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2. Gelbar NW, Smith I, Reichow B. {{Systematic Review of Articles Describing Experience and Supports of Individuals with Autism Enrolled in College and University Programs}}. {J Autism Dev Disord};2014 (May 11)
The increase in the number of higher-functioning individuals with autism spectrum disorders (ASD) is likely to lead to an increased interest in postsecondary opportunities including degree-granting college and university programs. To provide an understanding of the current evidence-base for supporting individuals with ASD in higher education, this article reports the results of a systematic review of the literature concerning college students with ASD. Overall, 20 articles describing 69 individuals met the inclusion criteria. This small number of articles and participants indicates the scarcity of research on this topic and only two of these studies were experimental in nature. These studies described a video-self modeling intervention and a counseling intervention respectively. Eighteen « case studies » were also present in the literature that described difficulties ranging from anxiety to housing concerns. This review deliniates the limitation of our understanding of effective college programming for individuals with ASD.
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3. Smith CB, Liu Z. {{Lithium: A Promising Treatment for Fragile X Syndrome}}. {ACS Chem Neurosci};2014 (May 11)
Fragile X syndrome is an inherited disorder that results in intellectual disability and a characteristic behavioral profile that includes autism spectrum disorder, attention deficit hyperactivity disorder, sensory hypersensitivity, hyperarousal, and anxiety. The silencing of FMR1 and the consequent absence of its protein product, FMRP, is the most common cause of fragile X. The development of animal models of fragile X syndrome 20 years ago has produced a considerable increase in our understanding of the consequences of the absence of FMRP on the structure and function of the nervous system. Some of the insights gained have led to proposals of treatment strategies that are based on cellular and molecular changes observed in animals lacking FMRP. One such proposal is treatment with lithium, a drug with a long history of clinical efficacy in psychiatry and a drug with newly described uses in degenerative disorders of the nervous system. Lithium treatment has been studied extensively in both mouse and fruit fly models of FXS, and it has been shown to reverse numerous behavioral, physiological, cellular, and molecular phenotypes. A report of a pilot clinical trial on a limited number of adult FXS patients indicated that measureable improvements in behavior and function were seen after two months of lithium treatment. A double-blind clinical trial of lithium treatment in FXS patients is now needed.
