Nanometer‐thick 2D carbon structures (“carbon nanosheets”) are processed from commercially available expanded graphite. These carbon nanosheets are then incorporated in various polymers to produce flexible nanocomposites that exhibit record‐setting anisotropic thermal conductivities, which may prove highly valuable in many technological applications.
Duchenne Muscular Dystrophy (DMD) is a neurogenetic developmental disorder that presents with progressive muscular weakness. It is caused by a mutation in a gene that results in the absence of specific products that normally localize to muscle cells and the central nervous system (CNS). The majority of affected individuals have IQs within the normal range, generally with lower verbal than performance IQ scores. Prior work has demonstrated selective deficits on tests of verbal span and immediate memory. For the current study, 26 boys with DMD (and normal intellectual function) and their unaffected siblings were evaluated. Paired comparisons demonstrated that the children with DMD had significantly poorer academic achievement scores than their siblings, even though their vocabulary levels and home and educational environments were comparable. Children with DMD also had more behavioral concerns, physical disabilities, and poorer verbal memory spans. Linear regression indicated that behavioral concerns, executive function, and physical disability did not contribute substantially to academic performance, whereas performance on verbal span did. DMD presents with a selective developmental aberration in verbal span that has wide-ranging consequences on learning skills.Duchenne Muscular Dystrophy (DMD) is a neurogenetic developmental disability. DMD is known mainly as a genetic disease of muscle, although it also has developmental consequences on the central nervous system. DMD is caused by a mutation of a gene on the X chromosome (Koenig et al., 1987). It occurs in about 1/3,200 boys and results in progressive muscular weakness. DMD is the most common fatal childhood inherited disorder, and affected individuals rarely live past their late -20s. NIH-PA Author ManuscriptNIH-PA Author Manuscript NIH-PA Author ManuscriptEver since its original characterization by Duchenne (1872), boys with DMD have been known to be at increased risk of mental retardation, although the majority of boys with DMD are not mentally retarded. Whether impaired cognitive function in individuals with DMD resulted from the stresses of living with a chronic, progressive, physically disabling disorder or from the underlying etiology affecting the development of the nervous system was uncertain. Yet, well before discovery of the gene that causes the disorder, decreased academic achievement scores were documented, prompting investigators to write that "an investigation of intelligence and academic achievement should be a routine part" of care for all children with DMD (Worden & Vignos, 1962).The discovery that the mutated gene in DMD codes for multiple protein products that localize to separate tissue types, including muscle and brain, offers a potential explanation for the cognitive manifestation of the DMD phenotype (for reviews see J. L. Anderson, Head, Rae, & Morley, 2002;Mehler, 2000). In the brain, dystrophin isoforms normally localize to circumscribed cerebral and cerebellar cortical regions and are absent in autopsied brains of individu...
The goal of the current investigation was to examine adaptive behavior and cognitive skills in young children with Duchenne muscular dystrophy (DMD), a genetic disorder that causes progressive muscular weakness and concomitant cognitive deficits. Previous studies have documented specific language deficits in older children with DMD, but there are limited data on younger children. Twenty children with DMD who were between 3 and 6 years old and 20 unaffected family control children were recruited. Parents completed questionnaires relating to development and adaptive functioning, while children completed neuropsychological testing. Results of paired t tests indicate that children with DMD are rated as delayed relative to familial controls on measures of adaptive functioning, as assessed by the Vineland Adaptive Behavior Scales. Furthermore, children with DMD exhibit impairments on multiple measures of cognition, including measures of receptive language, expressive language, visuo-spatial skills, fine-motor skills, attention, and memory skills. Across all domains examined, the young children with DMD performed more poorly than their familial controls. These deficits appear to be more generalized than those reported in older children with this disorder. Dystrophin, a missing protein product, is hypothesized to be responsible for these cognitive and behavioral impairments. (JINS, 2008, 14, 853-861.)
Duchenne muscular dystrophy (DMD) is a chronic, progressive pediatric disease that affects both muscle and brain. The objectives of the study were to examine parent reported behavior in children with DMD, investigate the influence of chronic illness, intellectual ability and etiology on behavior, and determine whether a specific behavioral profile is associated with DMD. Parental ratings of boys with DMD (n = 181) on the Child Behavior Checklist behavior scales were examined and compared to reported findings of children with other chronic illnesses, unaffected siblings of boys with DMD (n = 86), and children with cerebral palsy (CP) (n = 42). Increased ratings of general behavior problems were reported, and neither physical progression nor intellectual level contributed to behavioral ratings. Among the children with DMD, the Social Problem behavior scale had the greatest number of "clinically significant" ratings (34%). Between-group comparisons showed significantly more boys with DMD were rated as having social behavior problems than either the sibling or CP comparison groups. In addition to the increase in reported behavioral problems likely related to the effects of chronic illness, boys with DMD may be at heightened risk for specific social behavior problems. The specificity of the findings of the behavior profile in DMD may be partially due to the lack of dystrophin isoforms in the central nervous system, and not solely a reactive response to the illness.
In addition to the commonly reported delays in motor milestones, the current study documents delays in the acquisition of language milestones as well. These early delays are associated with significant impairments in later cognitive functioning.
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