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OBJECTIVE: The current study examined whether the arithmetical calculation skills of children, adolescents, and young adults with isolated cleft of the lip and/or palate (iCL/P) differ significantly from unaffected control participants. Comparisons of potential neuropsychological predictors of arithmetical calculation were also conducted to determine whether these variables differ significantly for participants with iCL/P. METHOD: Participants (N = 176; 93 iCL/P and 83 Control) ranged in age from 7 to 26 years old. A standardized battery of achievement and neuropsychological skills was administered. Between group differences on math achievement was assessed through a univariate analysis of covariance. Relationships between neuropsychological measures and math achievement were analyzed separately for participants with iCL/P and controls through hierarchical linear regressions. RESULTS: Arithmetical calculation was significantly lower for the iCL/P group. Rapid naming, sustained attention, and visual-spatial organization were significant predictors for the iCL/P group; rapid naming was the lone variable that was significantly more predictive of arithmetical calculation for the iCL/P group than for control participants. CONCLUSIONS: These results suggest that inefficient verbal label retrieval related to short-term memory (STM) deficits underlie the calculation difficulties of individuals with iCL/P. These findings have implications for approaches to remediation, as well as future research. (PsycINFO Database Record

Huntington disease is a neurodegenerative disorder caused by a gene (HTT) with a unique feature of trinucleotide repeats ranging from 10 to 35 in healthy people; when expanded beyond 39 repeats, Huntington disease develops. Animal models demonstrate that HTT is vital to brain development; however, this has not been studied in humans. Moreover, evidence suggests that triplet repeat genes may have been vital in evolution of the human brain. Here we evaluate brain structure using magnetic resonance imaging and brain function using cognitive tests in a sample of school-aged children ages 6 to 18 years old. DNA samples were processed to quantify the number of CAG repeats within HTT. We find that the number of repeats in HTT, below disease threshold, confers advantageous changes in brain structure and general intelligence (IQ): the higher the number of repeats, the greater the change in brain structure, and the higher the IQ. The pattern of structural brain changes associated with HTT is strikingly different between males and females. HTT may confer an advantage or a disadvantage depending on the repeat length, playing a key role in either the evolution of a superior human brain or development of a uniquely human brain disease. © 2016 Wiley Periodicals, Inc.

Huntington disease (HD) is a neurodegenerative disorder that involves preferential atrophy in the striatal complex and related subcortical nuclei. In this article, which is based on a dataset extracted from the PREDICT-HD study, we use statistical shape analysis with deformation markers obtained through "Large Deformation Diffeomorphic Metric Mapping" of cortical surfaces to highlight specific atrophy patterns in the caudate, putamen, and globus pallidus, at different prodromal stages of the disease. On the basis of the relation to cortico-basal ganglia circuitry, we propose that statistical shape analysis, along with other structural and functional imaging studies, may help expand our understanding of the brain circuitry affected and other aspects of the neurobiology of HD, and also guide the most effective strategies for intervention.

Abstract Background A detailed behavioral profile associated with focal congenital malformation of the ventromedial prefrontal cortex (vmPFC) has not been reported previously. Here we describe a 14 year-old boy, B.W., with neurological and psychiatric sequelae stemming from focal cortical malformation of the left vmPFC. Case Presentation B.W.'s behavior has been characterized through extensive review Patience of clinical and personal records along with behavioral and neuropsychological testing. A central feature of the behavioral profile is severe antisocial behavior. He is aggressive, manipulative, and callous; features consistent with psychopathy. Other problems include: egocentricity, impulsivity, hyperactivity, lack of empathy, lack of respect for authority, impaired moral judgment, an inability to plan ahead, and poor frustration tolerance. Conclusions The vmPFC has a profound contribution to the development of human prosocial behavior. B.W. demonstrates how a congenital lesion to this cortical region severely disrupts this process.

The purpose of this study was to assess neurological soft signs in children and adolescents with isolated cleft of the lip or palate (iCL/P) compared with healthy controls. Children with iCL/P were recruited through the university cleft clinic. Control subjects were recruited through advertisements. Of the 166 subjects who participated (age range 7 to 17 years, M = 12.5, SD = 3.2), 77 had iCL/P (48 male) and 89 were healthy controls (44 male). All participants took the Physical and Neurological Examination of Subtle Signs and selected tests of motor coordination. A multivariate analysis of variance assessed differences between subjects with and without iCL/P. Also, a Pearson correlation estimated the relationship between the neurological soft signs and age. Subjects with iCL/P scored significantly higher on all neurological soft signs variables. These higher scores were associated with younger age. Findings lend support to the hypothesis of aberrant brain development in children with iCL/P.