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Varieties of functional deficits in prosopagnosia.

Prosopagnosia is a neurologically based deficit characterized by the inability to recognize faces of known individuals in the absence of severe intellectual, perceptual, and memory impairments. The nature of the underlying disturbance was investigated in three patients in an attempt to identify the structural and functional levels at which the processing of faces breaks down, the relation between prosopagnosia and associated deficits, and the specificity of the prosopagnosic disturbance. The breakdown of face processing resulted from unilateral damage in different cerebral structures of the right hemisphere in the three patients, and it involved different functional levels of face processing, but all three patients displayed perceptual impairments of unequal severity. In one patient (R.M.), the deficit encompassed all perceptual operations on faces, including matching identical views of the same faces, but it did not extend to all categories of objects characterized by a close similarity among their instances; the second patient (P.M.) exhibited a less severe perceptual impairment but was unable to derive the configurational properties from a facial representation and to extract its physiognomic invariants; the third patient (P.C.) had not lost the capacity to differentiate faces on the basis of their configurations but could not associate a facial representation with its pertinent memories. Associated deficits were present in each patient but differed depending on the anatomofunctional locus of the breakdown, although all patients were impaired at recognizing noncanonical views of objects that they readily recognized when shown from a conventional viewpoint. However, performance dissociation within patients and double dissociation between patients suggest that these associated deficits are not necessary concomitants of prosopagnosia.

Agnosia↗

[A case report of pyridoxine-responsive homocystinuria].

INTRODUCTION: Homocystinuria is a rare, congenital, autosomal-recessive, metabolic disease biochemically characterized by homocysteinemia and homocystinuria and by multi-system clinical disorders. It is a biochemical abnormality of methionine metabolism caused either by transulfuration pathway disorders or by disorders of homocysteine remethilation into methionine and as such it can be a result of numerous specific and different genetic lesions. CASE REPORT: Homocystinuria is most commonly caused by deficiency of cystationine beta synthetase enzyme which catalyses the synthesis of cystathionine from homocysteine and serine in the methinione pathway. This results in accumulation of homocysteine and methionine in plasma and leads to excretion of excessive urinary homocysteine. Depending on specific property of the mutant enzyme molecule some patients respond to very high doses of pyridoxine with decreases of methionine and homocystine and some not. Pyridoxine responsiveness is based on the presence of small residual activity of cystathionine beta synthetase which is not present in nonresponsive patients. Homocystinuria due to cystathionine beta-synthase (CBS) deficiency is characterized by numerous different clinical abnormalities, but changes in four organ systems are dominant (eye, skeletal, central nervous and vascular system). CASE DESCRIPTION: Six years ago a six year-old boy was admitted to the hospital with vision problems. Ophthalmologic examination revealed a lens dislocation and because of many stigmates the child was sent to endocrinologist. The child had a marfanoid stature, with pectus carinatum and genu valgum, ataxic gait with motoric discoordination, muscle tone which ranged from hypotonia to hypertonia of extrapvramidal type and low intellectual abilities. A simple cyanide-nitroprusside test of patient's urine was positive suggesting homocystinuria. The diagnosis was established after plasma and urine amino acid analysis by HPLC. Concentration of homocystine and methionine were 52 mumol/l and 57 mumol/l in plasma and 249 mumol/du and 55 mumol/du in urine, respectively. After four months of treatment with pyridoxine there were not any significant changes in plasma homocysteine and methionine, but at the same time decrease in urine of these two amino acids were more than 2.5 times higher. This confirms that the patient has a pyridoxine-responsive type of homocystinuria and the dose was increased to 60 mg/day and 600 mg/day. This results in further decline of homocysteine and methionine in plasma and urine which persists up to now (for six years).

Child↗

Identifying the educative and suppressive effects of positive practice and restitutional overcorrection.

Two major features of the overcorrection procedure, restitution and positive practice, were analyzed for their educative and suppressive properties in the treatment of profoundly retarded adults. Positive practice techniques that were topographically similar and dissimilar to the target behavior were studied. Eating behavior and puzzle performance were observed. Restitutional overcorrection and both forms of positive practice were effective for suppressing inappropriate behaviors. Even when appropriate behaviors had been acquired by positive practice, restitution and dissimilar positive practice were generally ineffective for increasing their rate of occurrence. However, topographically similar positive practice was successful as a means of teaching new appropriate behaviors.

Adult↗

Reliability of the Matson Evaluation of Social Skills in Individuals with Severe Retardation (MESSIER).

Recently, the concepts of social competence and social skills have become important aspects of the evolving definitions of mental retardation. However, no psychometrically sound instruments exist for assessing social skills in lower functioning developmentally disabled people. This study examined the psychometric properties of the Matson Evaluation of Social Skills for Individuals with Severe Retardation (MESSIER)--a new scale designed to measure social skills in adults with severe developmental disabilities. The authors conducted a preliminary evaluation of the test-retest and interrater reliability of the MESSIER. It was determined that the MESSIER has high stability across raters and good stability over time. In addition, good internal consistency was established with coefficient alpha. Potential uses for the scale and directions for future research are discussed.

Adult↗

Treatment of self-injurious behavior using a water mist: initial response suppression and generalization.

This study evaluated the effects of a fine mist of water applied to the face contingent upon self-injurious behavior (SIB) exhibited by profoundly retarded persons. In Experiment 1, results of individual reversal designs showed substantial reductions in a variety of SIB's (mouthing, hand biting, skin tearing, and head banging) for seven participants. In Experiment 2, two participants who frequently bit their hands were each observed in two different settings. Following initial baselines in each setting, a series of manipulations was undertaken to compare the effects of mild verbal punishment ("No") with those of a combined treatment ("No" plus mist procedure). Results in one setting indicated that "No" suppressed SIB only after it was first paired with the water mist. Data also suggested that, once acquired, the punishing properties of "No" could be extended to a second setting in which the mist was never applied, and that these effects could be generalized across therapists. Results of these experiments indicate that the water mist procedure may be an effective alternative to traditional punishment techniques. Although conclusions regarding generalization are limited due to the brevity of the maintenance conditions, the data suggest that treatment gains may be transferred to more acceptable forms of social punishment and reinforcement.

Adolescent↗

Mentally retarded criminal offenders in Denmark.

Based on data from the Danish Central Criminal Register, the total number of mentally retarded offenders serving statutory care orders on a census day decreased from 290 in 1973 to 91 in 1984. The reduction was caused by shorter sentences and a dramatic decrease in the number of sentenced borderline retarded offenders: the total number of sentences per year slightly decreased, and the number of first-time sentences was stable. Crimes of property are decreasing among this population, while violence, arson, and sexual offences are increasing. Behaviour disorder was found in 87.5% of 91 offenders serving care orders in 1984. Offensive behaviour was significantly predicted by early institutionalisation, having retarded or divorced parents of low socio-economic status, and behaviour disorder of social-aggressive type. Independent significant effects were attributed to behaviour disorder and low socioeconomic background. All predictors were closely correlated. Biological factors did not have any significant predictive value.

Adolescent↗

Sensory reinforcement: effects of response-contingent vestibular stimulation on multiply handicapped children.

The reinforcing properties of vestibular stimulation were investigated, including the relative preference for vestibular stimulation and food, praise, visual, and auditory stimulation. Five multiply handicapped, severely retarded young children were trained to operate a pressure-sensitive adaptive switch to gain access to a given reinforcer during successive intervention phases. The results suggest that vestibular stimulation was reinforcing to all subjects. Vestibular stimulation appeared to be the preferred reinforcer for 4 of the 5 children. Results were discussed relative to the movement deprivation experienced by nonambulatory children. Possible implications for programming efforts were described.

Child↗

Exploration of psychometric properties of the developmental behavior checklist.

The Developmental Behavior Checklist (DBC; Einfeld & Tonge, 1995) is one of a very small number of psychopathology assessment instruments designed specifically for use with children and adolescents with mental retardation. In the present study, a factor analysis of the DBC was performed using a sample of 531 children and adolescents. This analysis revealed a factor structure sharing a good deal of overlap with the factor analyses of the DBC's developers. Furthermore, the high levels of internal consistency of the DBC subscales were replicated. Further research and development is needed on the DBC and other instruments for children and adolescents with mental retardation.

Adolescent↗

Validation of tardive dyskinesia as measured on the Dyskinesia Identification System--Coldwater.

Identification and description of tardive dyskinesia have been hampered by the absence of systematic studies assessing the psychometric properties of existing instruments. The purpose of this study was to establish construct validation of one of these instruments, the Dyskinesia Identification System--Coldwater. Cross-sectional prevalence assessment data of 519 institutionalized mentally retarded persons were used. The 34 items of this instrument were subjected to exploratory factor analysis. A six-factor orthogonal solution was selected. Multiple regression analyses supported the established relationships of demographic variables, such as age and sex, with the tardive dyskinesia scores generated by the instrument.

Adolescent↗

The development of speech act comprehension in mentally retarded individuals and nonretarded children.

Understanding the speech act(s) a sentence conveys requires that a listener follow conversational rules and use clues provided by the sentence and the context in which it is spoken. To trace the development of speech act comprehension in mentally retarded and nonretarded school age children, we examined their responses to sentences such as Would you open the telephone book? and Could you roll the shoebox? These sentences can be intended as questions or as directives. Nonretarded adults follow the "answer obviousness" rule and interpret a sentence of this type as a question if the answer to the question is nonobvious to the speaker, but as a directive if the question has an obvious answer. We manipulated answer obviousness by varying both the context and linguistic properties of the interrogative. Retarded and nonretarded individuals at the nonverbal MAs of 5, 7, and 9 years were studied. Retarded and nonretarded individuals at all MAs followed the answer obviousness rule and used the contextual and linguistic clues available. There were only minor differences between MA-matched retarded and nonretarded subjects despite the fact that the retarded subjects had serious deficits in receptive linguistic competence. The implications for understanding the development of comprehension in retarded persons and for understanding the relations among language, cognition, and communication in general are considered.

Child↗

Psychometric properties of ADHD rating scales among children with mental retardation.

The validity of hyperactivity rating scales in children with mental retardation was evaluated. Forty-eight children with mental retardation were rated by parents, teachers and teaching assistants on rating scales measuring Attention Deficit/Hyperactivity Disorder (ADHD) as part of a related investigation. In addition, direct observations were conducted using the Abikoff Classroom Observation Code. The concurrent validity of each scale was examined. Scales completed by both teachers and teaching assistants were found to provide valid information for the assessment of ADHD in mentally retarded children. Results provided the best support for the ABC-C in the assessment of ADHD in mentally retarded children.

Attention Deficit Disorder with Hyperactivity↗

Lost in translation: a genealogy of the "social capital" concept in public health.

STUDY OBJECTIVE: To examine the genealogy of the social capital concept in public health, with attention to the epistemological concerns and academic practices that shaped the way in which this concept was translated into public health. DESIGN: A citation-network path analysis of the public health literature on social capital was used to generate a genealogy of the social capital concept in public health. The analysis identifies the intellectual sources, influential texts, and developments in the conceptualisation of social capital in public health. PARTICIPANTS: The population of 227 texts (articles, books, reports) was selected in two phases. Phase 1 texts were articles in the PubMed database with "social capital" in their title published before 2003 (n = 65). Phase 2 texts are those texts cited more than once by phase 1 articles (n = 165). MAIN RESULTS: The analysis shows how the scholarship of Robert Putnam has been absorbed into public health research, how three seminal texts appearing in 1996 and 1997 helped shape the communitarian form that the social capital concept has assumed in public health, and how both were influenced by the epistemological context of social epidemiology at the time. CONCLUSIONS: Originally viewed in public health research as an ecological level, psychosocial mechanism that might mediate the income inequality-health pathway, the dominance of the communitarian approach to social capital has given disproportionate attention to normative and associational properties of places. Network approaches to social capital were lost in this translation. Recovering them is key to a full translation and conceptualisation of social capital in public health.

Health Services Research↗

On the displacement of leisure items by food during multiple-stimulus preference assessments.

Previous studies have demonstrated that when food and leisure stimuli are combined in multiple-stimulus preference assessments, individuals typically select food more often, although the leisure stimuli also have known reinforcing properties. The purpose of the current study was to replicate this effect and determine its durability by examining the effect after mealtimes. Four adults who had been diagnosed with severe mental retardation were given three initial multiple-stimulus (without replacement) preference assessments (i.e., food, leisure stimuli, and combined). All participants selected food items as the most preferred stimuli in the combined assessments. Combined assessments were then administered immediately before and after the evening meal for each participant for 1 week. The results showed similar data both before and after mealtimes.

Adult↗

A survey of the criminal careers of restriction order patients.

The offenders committed to local hospitals in 1962 and 1964 under a hospital order with restriction of discharge were followed up for 15 years with regard to subsequent court appearances, hospital admissions, and death. One hundred and forty-six were traced. Of these, 49 per cent had been diagnosed as mentally ill: most of this group were committed for violent offences. Thirty-five per cent were mentally subnormal: they were frequently committed for sexual offences, arson and property offences. Twelve per cent were diagnosed psychopathic: this group had a wide variety of committal offences. Fourteen per cent of the mentally ill and 17 per cent of the mentally subnormal were still in hospital after 15 years, but 51 per cent of the mentally ill and 36 per cent of the psychopaths were released within two years. Few of the subsequent offences were serious in any of the diagnostic groups. Since 1973 there has been a dramatic fall in restriction orders, especially for mentally subnormal patients.

Adult↗

Reliability and validity of the interview and self-report versions of the BASIS-32.

The psychometric properties of the interview and self-report versions of the BASIS-32 were compared. A total of 120 severely mentally ill adults enrolled in psychosocial rehabilitation were randomly assigned to either a self-report or an interview condition. The BASIS-32 had good internal consistency and test-retest reliability on most subscales; coefficients were higher in the self-report condition. Only the interview version of the psychosis subscale had unacceptable internal consistency. Validity correlations were generally good for the symptom subscales but disappointing for the functional domains. The subscale scores did not discriminate between diagnostic subgroups.

Adult↗

Idiocy and the law in colonial New England.

A review of the laws and records of the courts of colonial New England indicate some ways the early settlers thought about and responded to idiocy. Early Massachusetts laws extended certain rights to idiots: They authorized the transfer of property, exonerated idiots who committed capital crimes, and extended relief to idiots who were impoverished. There is no documentation of the implementation of these laws nor is there direct reference to idiocy in court proceedings. Nevertheless, the court records identify certain individuals with incompetence and atypical behavior suggestive of idiocy. Most of the colonial laws as well as the colonists' ways of thinking about idiocy originated in English common law and custom. The New England colonial laws and records of the courts offer insights into contemporary issues regarding mental retardation.

Criminal Law↗

Hierarchical assessment of social competence.

Three properties associated with the Test of the Hierarchy of INductive Knowledge (THINK) were examined, namely, that the processes assessed by the measure are (a) cumulative and hierarchical, (b) developmental, and (c) related to behavioral criteria. Analyses of data collected from 120 EMR children between the ages of 9 and 14 years generally support the developmental and criterion-related features of the measure, but only partially confirm the hierarchical component. Discussion of the results was focused on the emphasis necessary in subsequent empirical work and the measure's potential for instructional applications in relation to current assessment issues in special education.

Adolescent↗

Fragile X expression and X inactivation. I. The expression of the fragile site at Xq27.3 is not suppressed on inactive X chromosomes separated from the active homologue.

The inactive fragile X chromosomes of a 47,fra(X),fra(X),Y male with a typical fragile X phenotype were successfully separated from the active homologues by means of somatic cell hybridization. It was shown by FUdR-induction and caffein-posttreatment that the separated inactive X chromosomes expressed their fragile sites and that the presence of an active mutated X chromosome was not a prerequisite for fragile X expression. The fragility seems to be an intrinsic property of the individual fragile site. This result is in favour of the classical concept that the fragile site at Xq27.3 has a primary pathogenetic function in this syndrome, although the fragility itself could represent a secondary phenomenon related to an unknown alteration of the DNA in this chromosome region. It is also concluded that inactivation of the fragile X chromosome in females is not responsible for either false negative fragile X findings or the observation of fragile X negative colonies isolated from fragile X positive fibroblasts in heterozygotes.

Chromosome Banding↗