Practical Approach to Longitudinal Neurologic Care of Adults With X-Linked Adrenoleukodystrophy and Adrenomyeloneuropathy.

Although X-linked adrenoleukodystrophy (ALD) has historically been considered a childhood disease managed by pediatric neurologists, it is one of the most common leukodystrophies diagnosed in adulthood. An increase in both male and female adults reaching diagnosis due to familial cases identified by state newborn screening panels and more widespread use of genetic testing results in a large cohort of presymptomatic or early symptomatic adults. This population is in urgent need of standardized assessments and follow-up care.

Context influences preference for and level of physical activity of adolescents with intellectual and developmental disabilities.

The purpose of the current study was to replicate and extend Hustyi, Normand, Larson, and Morley (2012) by determining the effects of different contexts on physical activity displayed by adolescents with intellectual and developmental disabilities, and determining preference for various activities. Results indicated that an exergaming condition produced the highest levels of activity. Results of a preference assessment indicated that 2 out of 3 participants preferred the physical activity context to the sedentary.

Functional analysis and treatment of problem behavior related to mands for rearrangement.

This study is a systematic replication of a functional analysis (FA) of the relation between mands and problem behavior. We extended treatment approaches for this problem behavior function, and describe the treatment of problem behavior related to mands for rearrangement demonstrated by a 12-year-old girl with autism spectrum disorder and Smith-Magenis syndrome. The mands consisted of requests for others to change their body positioning or proximity, or rearrange items back to their original position.

Noncontingent reinforcement for the treatment of severe problem behavior: An analysis of 27 consecutive applications.

Noncontingent reinforcement (NCR) is a commonly used treatment for severe problem behavior displayed by individuals with intellectual and developmental disabilities. The current study sought to extend the literature by reporting outcomes achieved with 27 consecutive applications of NCR as the primary treatment for severe problem behavior. All applications of NCR were included regardless of treatment outcome to minimize selection bias favoring successful cases.

Telomere longitudinal shortening as a biomarker for dementia status of adults with Down syndrome.

Previous studies have suggested that Alzheimer's disease (AD) causes an accelerated shortening of telomeres, the ends of chromosomes consisting of highly conserved TTAGGG repeats that, because of unidirectional 5'-3' DNA synthesis, lose end point material with each cell division. Our own previous work suggested that telomere length of T-lymphocytes might be a remarkably accurate biomarker for "mild cognitive impairment" in adults with Down syndrome (MCI-DS), a population at dramatically high risk for AD. To verify that the progression of cognitive and functional losses due to AD produced this observed telomere shortening, we have now examined sequential changes in telomere length in five individuals with Down syndrome (3F, 2M) as they transitioned from preclinical AD to MCI-DS (N = 4) or dementia (N = 1).

The safety of functional analyses of self-injurious behavior.

Functional analysis is the most precise method of identifying variables that maintain self-injurious behavior (SIB), and its use may lead to more effective treatment. One criticism and potential limitation of a functional analysis is that it may unnecessarily expose individuals to a higher risk of injury (Betz & Fisher, 2011). The purpose of this study was to determine if there were higher levels and severity of injury during the functional analysis than outside the functional analysis.

Identification of Emerging Self-Injurious Behavior in Young Children: A Preliminary Study.

Self-injurious behavior (SIB) is a chronic disorder that often begins in early childhood; however, few studies have examined the onset of SIB in young children. This preliminary study reports on the identification, assessment and observation of SIB in 32 children who had begun to engage in SIB within the previous 6 months. Participants were ages birth to 5 years and presented with or were at risk for intellectual and/or developmental disabilities.

Brain derived neurotrophic factor and serotonin transporter binding as markers of clinical response to fluoxetine therapy in children with autism.

Fluoxetine, a selective serotonin reuptake inhibitor (SSRI), has shown favorable effects in some children with autism. There are no previous studies evaluating the connection between clinical outcome and markers of clinical response to fluoxetine treatment. We examined serum brain derived neurotrophic factor (BDNF) concentrations and serotonin transporter (SERT) binding in the medial frontal cortex and midbrain, measured by single photon emission computed tomography (SPECT) scanning, in a group of 13 autistic children and adolescents (12 males, one female; age 5-16 years), who were treated for six months with fluoxetine at a dose range of 10-40 mg/day.

Concurrent reinforcement schedules for problem behavior and appropriate behavior: experimental applications of the matching law.

This study evaluated how children who exhibited functionally equivalent problem and appropriate behavior allocate responding to experimentally arranged reinforcer rates. Relative reinforcer rates were arranged on concurrent variable-interval schedules and effects on relative response rates were interpreted using the generalized matching equation. Results showed that relative rates of responding approximated relative rates of reinforcement.

Consistent visual analyses of intrasubject data.

Visual inspection of single-case data is the primary method of interpretation of the effects of an independent variable on a dependent variable in applied behavior analysis. The purpose of the current study was to replicate and extend the results of DeProspero and Cohen (1979) by reexamining the consistency of visual analysis across raters. We recruited members of the board of editors and associate editors for the Journal of Applied Behavior Analysis to judge graphs on a 100-point scale of experimental control and by providing a dichotomous response (i.

Sex and the pathogenesis of cerebral palsy.

Cerebral palsy (CP) and related developmental disorders are more common in males than in females, but the reasons for this disparity are uncertain. Males born very preterm also appear to be more vulnerable to white matter injury and intraventricular hemorrhage than females. Experimental studies in adult animals and data from adult patients with stroke indicate that sex hormones such as estrogens provide protection against hypoxic-ischemic injury, and the neonatal brain is also influenced by these hormones.

Demyelination and remyelination after spinal cord injury.

Since the discovery in the 1960s that remyelination can occur in the damaged central nervous system (CNS) (Bunge et al. 1961), there has been much progress in understanding the cellular and molecular biology of oligodendroglia and the factors that regulate their propagation, migration, differentiation, maturation, and ability to myelinate nerve axons. More recently, greater understanding of disease states and the role of oligodendrocytes in remyelination have sparked tremendous interest in this once obscure field.

Teaching children with attention deficit hyperactivity disorder (ADHD) and autistic disorder (AD) how to swallow pills.

One barrier to medication adherence in pediatric populations is difficulty swallowing pills. Some children may not have prerequisite skills for pill swallowing, while others may have developed conditioned anxiety from repeated negative experiences. Eight children with attention deficit hyperactivity disorder or autistic disorder participated in behavioral training to increase cooperation with pill swallowing.

Educational neuroimaging: a proposed neuropsychological application of near-infrared spectroscopy (nIRS).

Objective: To provide a description of an emerging neuroimaging methodology, near-infrared spectroscopy (nIRS), and a potential educational application of the unique aspects of this technology.

Summary: nIRS is documented for its potential as a personal, portable, brain imaging system that may prove useful for cerebral monitoring in applied settings such as home, school, and work. The basis of nIRS brain imaging is reviewed, with summary descriptions of optical and neurovascular issues as well as a brief comparison to other brain imaging methodologies.

A method for identifying satiation versus extinction effects under noncontingent reinforcement schedules.

We evaluated one method for determining whether response suppression under noncontingent reinforcement (NCR) is a function of satiation or extinction. Three individuals with developmental disabilities who engaged in self-injurious behavior (SIB) or aggression participated. Results of functional analyses indicated that their problem behavior was maintained by social-positive reinforcement.

Comparison of single and multiple functional communication training responses for the treatment of problem behavior.

Two functional communication training (FCT) conditions without extinction were compared to treat the problem behavior of a child with developmental disabilities. The individual was taught to emit a single FCT response to obtain one of six items delivered in a randomized order or multiple FCT responses that specified the exact item. Results showed that only the FCT-multiple condition reduced problem behavior and maintained alternative mands.

A comparison of procedures for programming noncontingent reinforcement schedules.

We compared two methods for programming and thinning noncontingent reinforcement (NCR) schedules during the treatment of self-injurious behavior (SIB). The participants were 3 individuals who had been diagnosed with mental retardation. Results of functional analyses indicated that all participants' SIB was maintained by positive reinforcement (i.

The use of symmetrical "do" and "don't" requests to interrupt ongoing activities.

The results of a modified functional analysis demonstrated that aggression, displayed by a 27-year-old man, was occasioned by the use of "don't" requests to interrupt the client's ongoing and often inappropriate activities (e.g., lying on the floor, pica, inappropriate touching of others).