Cognitive assessment using the Rapid Assessment for Developmental Disabilities, Second Edition (RADD-2).

BACKGROUND: Individuals with developmental disabilities (DD) often have severe impairments and maladaptive behaviours that make it difficult to reliably assess their cognitive abilities. Given these challenges, the Rapid Assessment of Developmental Disabilities, Second Edition (RADD-2), was designed to measure general cognitive ability in this population. The purpose of this study is to demonstrate the battery's psychometric properties when used with individuals with DD who have challenging behavioural and psychiatric conditions and for those who have limited verbal skills. METHOD: The cognitive and adaptive behaviour skills of 193 children and adults with DD and considerable medical, behavioural and/or psychiatric problems were evaluated using the first and second editions of the RADD, Kaufmann Brief Intelligence Test - 2nd Edition, and Scales of Independent Behaviour - Revised Edition. Medication side effects and challenging behaviours were assessed using the Aberrant Behaviour Checklist. RESULTS: There were no floor or ceiling effects on the RADD-2. Both the nonverbal index and total scores had strong concurrent validity with other abbreviated tests of intellectual ability and good discriminant validity from measures of adaptive behaviour and medication side effects. RADD-2 scores also had strong criterion validity as they successfully differentiated between all levels of intellectual functioning. Age and sex did not differentially affect RADD-2 performance, and the co-occurrence of psychiatric conditions did not negatively affect performance. The only medical condition associated with lower RADD-2 performance was epilepsy. CONCLUSIONS: The RADD-2 can quantify the differential cognitive abilities of individuals with DD, even for those with minimal communication skills, challenging behaviours or severe medication side effects that can typically complicate assessment. This brief cognitive battery can be used to measure changes due to interventions, on the one hand, and progression of neurological disease, on the other.

The relationship between living arrangement and adherence to antiepileptic medications among individuals with developmental disabilities.

BACKGROUND: Non-adherence to antiepileptic drugs (AEDs) is associated with considerable morbidity and mortality in the general population but little is known about adherence in individuals with intellectual disability (ID). METHOD: Using the records of a closed pharmacy billing system over a 30 month period, we examined the medication non-adherence rates for AEDs among 793 individuals with ID. We calculated the medication possession ratio (number of days each participant was in possession of an AED), and defined non-adherence as 25% or more of the exposure days without the possession of an AED. All participants studied had filled prescriptions for AEDs spanning at least 6 months. RESULTS: Controlling for age and gender, we found non-adherence rates varied by living arrangement. Compared with those living in group homes, individuals with ID living in family homes or in semi-independent settings were significantly less adherent to AEDs (P < 0.0003). CONCLUSION: Non-adherence to AEDs is a potential medical risk for individuals with ID that is significantly impacted by the type of community living arrangement.

Recurrent mutations in DNAJC5 cause autosomal dominant Kufs disease.

We sought to identify the molecular basis of the autosomal dominant form of Kufs disease, an adult onset form of neuronal ceroid lipofuscinosis. We used a combination of classic linkage analysis and Next Generation Sequencing to map and identify mutations in DNAJC5 in a total of three families. We analyzed the clinical manifestations in 20 individuals with mutation in DNAJC5. We report here the mapping and the identification of a p.L116del mutation in DNAJC5 segregating with the disease in two distinct American families, as well as a p.L115R mutation in an additional family. The age of onset and clinical manifestations were very homogeneous among mutation positive individuals, including generalized tonic-clonic seizures, myoclonus, ataxia, speech deterioration, dementia, and premature death. A few individuals also exhibited parkinsonism. DNAJC5, which encodes the cysteine string protein (CSPα), a presynaptic protein implicated in neurodegeneration, causes autosomal dominant Kufs disease. The leucine residues at positions 115 and 116 are hotspots for mutations and result in a homogeneous phenotype of progressive myoclonus epilepsy with onset around 30 years old.

Rapid assessment of severe cognitive impairment in individuals with developmental disabilities.

BACKGROUND: Most standardized intelligence tests require more than 1 hour for administration, which is problematic when evaluating individuals with intellectual disabilities and developmental disabilities (IDDD), because a significant proportion of these individuals can not tolerate lengthy evaluations. Furthermore, most standardized intelligence tests are of limited usefulness for individuals with severe cognitive deficits because of floor effects. METHODS: A number of low-difficulty items were selected from standardized tests. A total of 271 participants with profound, severe, moderate and mild levels of cognitive impairment took part in this study. In the formative phase, 68 participants were evaluated with the selected items, and those items that differentiated between levels of cognitive impairment were retained in the battery. The instrument was then modified and standardized with an additional 203 participants. RESULTS: The instrument, referred to as the Rapid Assessment for Developmental Disabilities (RADD), required 10-25 min for administration. Internal reliability estimates from the RADD total score and from individual subtests satisfied conventional and rigorous statistical criteria (median alpha r = 0.93). The RADD total score was strongly correlated with the level of cognitive impairment (rho = 0.86). The RADD total score and individual subtests differentiated between all levels of cognitive impairment ( Wilks Lambda = 0.135, F(42,525.832) = 12.075, P < 0.001). Receiver operating characteristic curves indicated the instrument was particularly sensitive to the cognitive abilities of the most seriously impaired participants. CONCLUSIONS: The RADD, composed of low-difficulty items from published tests, is rapidly administered, assesses a wide range of cognitive skills and differentiates among all levels of cognitive impairment. The battery has clinical utility with populations exhibiting short attention spans because of its ability to quickly assess a wide range of cognitive abilities. The RADD also has research potential for the documentation of cognitive function in studies of individuals with IDDD.

Longitudinal prescribing patterns for psychoactive medications in community-based individuals with developmental disabilities: utilization of pharmacy records.

BACKGROUND: Little is known about longitudinal prescribing practices for psychoactive medications for individuals with intellectual disabilities and developmental disabilities (IDDD) who are living in community settings. METHODS: Computerized pharmacy records were accessed for 2344 community-based individuals with IDDD for whom a total of 3421 prescriptions were written during a 17-month period of study. Forty-two psychoactive medications were rank ordered in terms of prescription frequency. RESULTS: Fifty-two per cent (52%) of all prescriptions written during the study period were for psychoactive medications. Anticonvulsant, antipsychotic and antidepressant medications were the most commonly filled prescriptions among psychoactive medications. Sixty per cent (62%) of the study population was given prescriptions for more than one psychoactive medication and 36% received three or more psychoactive medications. During the study period there was a statistically significant increase in prescriptions filled for olanzapine, risperidone, valproic acid, and clonazepam whereas prescriptions filled for thioridazine, haloperidol, and benzotropine showed a significant decline (P < 0.05-0.001). Distribution of psychoactive drug class by age showed that the majority of prescriptions were filled for individuals between 20 and 50 years with the exception of prescriptions for psychostimulants which peaked for individuals prior to 20 years. CONCLUSIONS: (1) Analysis of pharmacy billing records provides a method for assessing prescribing patterns of psychoactive medications in community-based individuals with IDDD. (2) Polypharmacy for psychoactive medications is prevalent in this setting. (3) The second-generation antipsychotic medications are prominently represented by an increasing number of filled prescriptions during the study period.

Long-term effects of naltrexone on self-injurious behavior.

A subgroup of self-injuring patients responds positively to the opiate-blocking agent naltrexone in acute, double-blind studies. In this study we examined the effects of naltrexone after acute treatment and the long-term effects of naltrexone on SIB. Rates of SIB were collected from pretreatment baseline; a second baseline a year after the acute trial; and a subsequent 12-month double-blind, placebo-controlled treatment. A subgroup of patients decreased SIB for a year without treatment after acute exposure to naltrexone. Five participants who decreased SIB by 70% after acute treatment increased SIB to the long-term treatment with naltrexone. In contrast, those for whom SIB increased over the one-year treatment hiatus decreased their SIB after the first long-term treatment. Discussion of these complex effects considered the role of background opioid levels, dosing, and treatment regimen of naltrexone and other factors limiting receptor adaptation among patients who exhibit SIB.

Planned Activities Training for mothers of children with developmental disabilities. Community generalization and follow-up.

Planned Activities Training (PAT) teaches mothers to plan and structure activities to prevent challenging child behaviors. PAT was evaluated with four mothers of children with developmental disabilities, including autism, Down Syndrome, and ADHD. PAT was used independent of any other behavior management techniques to examine its impact on mother and child behaviors, which were examined in addition to "fidelity" data on the mothers' implementation of PAT techniques. A multiple probe experimental design across two families with a replication across two more families demonstrated that PAT produced marked improvements in mother and child behavior in three generalization settings. In most cases, mothers' use of PAT procedures more than doubled. Three mothers' appropriate behavior increased from 25% to 40%. Improvements in child behavior ranged from 20% to more than 50%. Intervention gains were maintained at 1, 3, and 6 months. These results suggest that PAT is a useful technique for promoting durable generalization of mother child skills.

Detection of Alzheimer disease in individuals with Down syndrome.

A comprehensive baseline of emotional functioning was established for adults with Down syndrome. Five emotional factors were studied using groups of (a) adults with Down syndrome (n = 30), (b) clinical control subjects with dementia of the Alzheimer type (n = 18), and (c) elderly control subjects without mental retardation (n = 25). Results of planned statistical comparisons showed indifference, pragnosia, and inappropriateness as primary emotional factors separating Down syndrome and Alzheimer disease groups from elderly control subjects without mental retardation. Indifference was also shown to covary with cognitive mental state, whereby increased levels of indifference were associated with decreased levels of cognitive functioning. The possibility of noncognitive variables signalling dementia of the Alzheimer type in individuals with Down syndrome was discussed.

An analysis of home-based oral rehydration therapy in the Kingdom of Lesotho.

Mothers in developing countries are being successfully taught to give an oral rehydration solution (ORS) at home. The quantity of oral rehydration fluid that mothers administer to their child remains a critical question. Inadequate quantities render oral rehydration therapy (ORT) ineffective. The primary focus of our investigation was direct measurement of the quantity of fluid unsupervised mothers gave their children at home. This study validates a methodology that provides precise information on ORS administration in the home, information essential for evaluating the effectiveness of home-based therapy and for planning programmes to influence unsupervised mothers to correctly administer ORS to their sick children. This study, conducted in the Kingdom of Lesotho, assessed the impact on home care of the national control of diarrhoeal disease (CDD) programme. Direct observations in the home established each child's status and the quantity of fluid used 24 hr after children left the ORT unit where mothers were instructed to give ORS at home. Data were gathered on natural consequences that might discourage use of ORS, such as vomiting, increased frequency of watery stools and distaste for the solution. The health status of most of the 197 children followed improved. The average volume of ORS administered was 544 ml. A full liter was given by 21% of the mothers. Only 3% of the mothers gave no ORS. The average dose was 65 ml/kg. Younger children got about the same volume as older children; thus, they received a higher ml/kg dose. Mothers gave more ORS to children who had more symptoms of diarrhoeal disease at the time they were brought to the clinic.(ABSTRACT TRUNCATED AT 250 WORDS)

Improving parent-child interactions for families of children with developmental disabilities.

Child Management Training (CMT) involves compliance training with a focus on consistent use of antecedents and consequences. Planned Activities Training (PAT) focuses on teaching parents to plan for and engage in activities with their children. A multiple probe design counterbalancing PAT and CMT showed that PAT and CMT were about equally effective in improving mother-child interactions in four families with children with developmental disabilities. Responses to a social validation questionnaire indicated that parents were satisfied with the services received, and that PAT was the slightly preferred treatment. Prior research demonstrated that PAT enhanced the results of CMT. The practical advantages of PAT over CMT are discussed.

Effect of naltrexone upon self-injurious behavior, learning and activity: a case study.

Naltrexone significantly attenuated self-injurious behavior in a 20-year-old mildly retarded autistic male patient. The patient was videotaped daily and behavior was evaluated with a time-sampling procedure. Behavioral ratings of SIB frequency, SIB severity, and activity were collected automatically with a computerized system. Learning and memory were tested on a weekly basis with a modification of a paired associate learning test (PALT). Treatment with naltrexone resulted in (a) attenuation of SIB in the unstructured setting and (b) improvements in learning and memory without influencing activity levels.

How much oral rehydration solution is actually administered during home-based therapy?

In some parts of the world up to one-half of all deaths in young children are attributable to dehydration associated with diarrhoea. As a countermeasure, mothers in underdeveloped countries are being successfully taught to give oral rehydration solution at home. There are, however, serious doubts as to whether mothers give their children enough. The focus of our investigation was a methodology capable of establishing the exact quantity of fluid administered by unsupervised mothers at home. Accurate quantitative data are essential for programme planning and evaluation. In our sample of 44 cases, only two children received more than 90 ml kg-1 day-1. The mean observed value was 44 ml kg-1 day-1 (SD 28.4); well below the recommended dosage. Preliminary data were also gathered on natural consequences which may discourage use of ORS such as vomiting, increased frequency of watery stools, and distaste for the solution.

The healthcom project and the behavioral management of diarrhea.

Sixteen million children die annually from causes which are largely preventable. Many of these deaths stem from malnutrition and diarrheal disease. This article presents a model of nutritional health and describes activities of the HEALTHCOM project related to nutritional health promotion and diarrheal disease control in developing countries. Specifically, HEALTHCOM includes a unique combination of communication and behavioral techniques for promoting health. One HEALTHCOM project took place in The Gambia where mass media and behavioral incentives proved a powerful combination in increasing mothers' knowledge of oral rehydration.

A focus on behavior: the role of health practices studies.

PIP: 90% of women in the Bangladesh Rural Advancement Committee (BRAC) rehydration therapy program learned the "Seven Points to Remember" about oral rehydration salts (ORS), but only 8% of women in some locations of the program area actually used ORS to treat diarrhea. Experimental analysis of behavior suggests 6 circumstances that may contribute to the absence of desirable behavior, either singly or in combination: necessary skills or knowledge may be absent; the ability to identify when to alter the behavior may be underdeveloped; necessary materials or implements may be unavailable, e.g., ORS packets often are out of stock; there may be no positive consequences for engaging in the behavior; there may be possible consequences for engaging in incompatible behavior, such as fasting during diarrhea; and there may be punishing consequences which discouraged the desired behavior pattern, e.g., a child may vomit or the diarrhea may appear to increase during rehydration. Behavior analysis is designed to identify the relevance of each of the 6 cited categories within the context of a specific culture, behavior, and/or individual. The behaviorist observes, questions, and tests behaviors, looking for the: cost to the individual of engaging in new practices; compatibility of new practices with existing patterns of behavior and cultural expectations; complexity of new practices; perceived and actual antecedents of a given practice; perceived and actual consequences of the practice, and observability of the target practice or its direct by-product. 2 examples help demonstrate how behavioral approaches such as careful observation and informal incentives can be applied to field programs. Some of the most salient principles emerging from recent behavioral studies include: observation of behavior within the broad context of the culture in which it is found; skillful arrangement of events so that reinforcement follows the desired behavior; individual record keeping or monitoring of behavior; and decisions of when and how to end a behavior change program should be systematic to ensure continued maintenance of the new practice. The practice of behavioral analysis does not substitute for the insights or methodologies of other disciplines. In fact, behavioral anaysis can help to enhance the contributions of other disciplines and sources of information by highlighting the contributions they have to offer.^ieng

A scatter plot for identifying stimulus control of problem behavior.

Line graphs that average response frequency over long periods obscure the major rate changes that indicate sources of behavioral control. A scatter plot can make patterns of responding identifiable and, in turn, suggest environmental features that occasion undesirable behavior. Use of scatter diagrams is illustrated in three cases.

Errorless learning: reinforcement contingencies and stimulus control transfer in delayed prompting.

Delayed prompting can produce errorless discrimination learning. There is inherent in the procedure a disparity in reinforcement density which favors unprompted over prompted responses. We used three schedules of reinforcement to investigate the impact of reinforcement probability on transfer of stimulus control. One schedule of reinforcement was equal prior to and following a prompt (CRF/CRF), the second favored unprompted responses (CRF/FR3), and the third favored responses following the prompt (FR3/CRF). Experimental questions concerned the probability of errors, the probability of transfer, and the rate of transfer in the context of delayed prompting. Transfer was accelerated when reinforcement probability favored anticipatory responding. The schedule that favored prompted responses did not prevent a shift to unprompted responding. Errors were infrequent across procedures. Reinforcement probability contributes to but does not entirely determine transfer of stimulus control from a delayed prompt.

Visual attention in retarded adults: combining stimuli which control incompatible behavior.

Eight severely retarded young men learned color and line-tilt discrimination. After 95% accuracy was achieved for both dimensions, they were combined to form "conflict-compound" stimuli in which prior reinforcement history was reversed for one element of the compound and unchanged for the other. When responding to the compound was 95% accurate, control exerted by each element was measured. The unchanged element consistently exercised control in agreement with the reinforcement contingencies associated with the compound, regardless of whether it was color or line orientation. The reversed element, which had a conflicting prior history or reinforcement, most often exerted control associated wtih original training, or no control, suggesting that it had been "ignored" during the compound. Conflict compounds produced selective attention. When elements were combined to form "compatible-compound" stimuli, both exercised control in agreement with the compound in post-tests. Selective attention was not produced by compatible compounds.

Withdrawal of chronic chlorpromazine medication: an experimental analysis.

Approximately 50% of all institutionalized, mentally retarded adults receive psychotropic medication to control inappropriate behavior. In this study, behaviors exhibited by five retarded adults were formally observed while they were on and off medication. Each subject had been receiving chlorpromazine for six or more years prior to the start of the study. The drug was withdrawn and readministered using a double-blind B-A-B (drug placebo-drug) design. Effects were highly individualized. Some desirable behavior emerged when chlorpromazine was discontinued.

Effects of diet and behavior therapy on social and motor behavior of retarded phenylketonuric adults: an experimental analysis.

The effects of a low phenylalanine diet on six retarded phenylketonuric adults were assessed. An ABA individual-subject design was used in experiment I to assess the effects of a low phenylalanine diet on social and motor behavior. Following a baseline during which the subjects ingested a normal phenylalanine diet (phase A), a low phenylalanine diet (phase B) was administered in a double blind fashion. Finally, the baseline condition (phase A) was reinstated (normal diet). The low phenylalanine diet resulted in few significant behavioral changes for those subjects with which proper methodologic controls were employed. However, for two of six subjects motor behavior, including stereotypy and tremor, seem to have ameliorated. In experiment II, applied behavior analysis techniques, including differential reinforcement of other behavior and time out, were combined to radically reduce the frequency of stereotypy and self-abuse exhibited by one of the six subjects of experiment I.