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Movement disorder in Down's syndrome: a possible marker of the severity of mental handicap.

This study examined the nature and prevalence of abnormal movements in adults with Down's syndrome and also the clinical correlates of orofacial dyskinesia and the relationship between dyskinesia and the level of functional and intellectual disability. Movement disorder, language age, and disability were assessed in an epidemiologically based sample of 145 individuals with Down's syndrome. Abnormal involuntary movements were common, with > 90% exhibiting dyskinesia, predominantly orofacial. Stereotypes were present in one-third of the sample. There was an association between the severity of dyskinesia and both current language age and functioning in terms of self-care and practical and academic skills, which suggested that dyskinesia may be a marker of the severity of mental handicap. The presence of dyskinesia was unrelated to neuroleptic exposure. Dyskinesia and stereotypies are very common in individuals with Down's syndrome and may represent an inherent manifestation of the disorder. The relationship between mental age and dyskinesia in Down's syndrome warrants further research.

Activities of Daily Living↗

Family stress and family strengths: a comparison of single- and two-parent families with handicapped children.

Using the Typology Model of Adjustment and Adaptation, a family stress model, the differences in family stressors, resources, family types, parental coping patterns, and child health indices were examined in 27 single-parent families and 27 two-parent families who had a child with cerebral palsy. The two groups of families were matched on the severity of the child's handicap and the age and gender of the parent. There was no significant difference between the two groups of families on the accumulation of stressors and demands, resource strains, family types, family cohesion, family resources of esteem/communication, mastery/health, extended family social support, and the child health indices of overall physical health status and health improvement. Single-parent families were significantly lower on financial well-being and the mother's coping related to maintaining family integration, cooperation, and optimism about the situation. Single-parent families also were higher on family adaptability than two-parent families, reflecting an ability to be more flexible in family rules, power structure, and role relationships.

Adaptation, Psychological↗

Handicaps not obligatory in sexual selection for resistance genes.

In previous models of parasite-driven sexual selection, the signals that reveal parasite resistances and that are used in mate choice should be costly to be reliable ("Handicap Principle"). However, sometimes parameter constellations could exist that allow for cheap signals directly revealing the identity of resistance genes. Despite the general conflict of interests between the sexes, sexual selection may, in these cases, have led males to focus on improving their offspring's survival chances instead of trying to get a maximum number of offspring. Males may achieve this by allowing choosy females to optimize costs and benefits of each resistance in the progeny. Multiple signals (such as odours) may serve to encode the necessary information about the identity of resistance genes. As an example, the connection between the major histocompatibility complex and odour-based mate choice in mice could be explained by this hypothesis.

Animals↗

The validity of the handicap principle in discrete action-response games

The validity of the handicap principle has spawned much debate in spite of the existence of a formal treatment. Simple models constructed to further investigate the issue were able both to prove and to disprove some of its claims. Here I show with the aid of a more general model, which takes into account both assumptions presented in these previous simple models: (1) that the previous results are not in conflict since they can be obtained as specific cases of this general model; (2) that ESS communication need not use costly signals, that is, even under conflict of interest, the cost of a signal used by a high-quality individual can be zero (or even negative) provided that the cost for low-quality signallers is high enough; (3) that only the cost relative to the benefits of the interaction should be higher for worse signallers; and (4) that in a discrete model the differential cost is only a necessary but not a sufficient condition for evolutionarily stable reliable communication. Copyright 1999 Academic Press.

Journal Article↗

Strategic choice handicaps when females seek high male net viability.

We examine a strategic-choice handicap model in which males send costly signals to advertise their quality to females. Females are concerned with the net viability of the male with whom they mate, where net viability is a function of the male's quality and signal. We identify circumstances in which a signaling equilibrium would require high-quality males to send signals so much larger than those of lower-quality males (to deter mimicry by the latter) as to yield lower net viabilities for the former. This causes females to shun males who send large signals, ensuring that there is no signaling equilibrium.

Animal Communication↗

Handicapped individua in evolutionary processes.

Usually, when considering an evolutionary development, the fittest individua and their successful mutations receive most attention. The less fit ones, existing due to selection-mutation equilibria, are regarded, as an inevitable waste. It appears that these handicapped individua play an important role in evolution, as they make escapes from evolutionary traps possible. It is concluded that crucial improvements come from mutations of mutants. The implications seem to account to some extent for the observed speed and mode of evolution. An appropriate stochastic model is introduced, numerical experiments reported and results discussed.

Biological Evolution↗

Covert dysphagia in the mentally handicapped: two case reports and a review of published literature.

Mentally handicapped patients with esophageal pathology may present a diagnostic challenge, as they may not complain of any specific symptoms or be able to give a clear history of either dysphagia or odynophagia. This paper reports 2 such patients who recently presented to the Royal Wolverhampton Hospitals. In the first case, upper gastrointestinal endoscopy under general anesthesia enabled both the identification and removal of a large esophageal foreign body. In the second case, repeated upper gastrointestinal endoscopy facilitated both the identification and nonsurgical management of a paraesophageal abscess.

Adult↗

Decreased bone density in severely handicapped children and adults, with reference to the influence of limited mobility and anticonvulsant medication.

Bone density and related biochemical parameters were investigated in institutionalised children and adults with severe handicaps, who were classified according to the degree of limited mobility (group 1, bed-ridden; group 2, capable of crawling; group 3, capable of walking) and according to whether or not they were receiving anticonvulsants. As determined by microdensitometric analysis of radiograms of the second metacarpal bone, bone width (D), bone pattern area (sigma GS) and bone salt density (sigma GS/D) were decreased in the patients, the decreases being most prominent in group 1, followed by groups 2 and 3, in that order. Significant decreases of sigma GS and sigma GS/D, but not of D, were found in patients on anticonvulsant treatment in comparison to patients without therapy. Serum alkaline phosphatase (Al-p) and parathyroid hormone (iPTH) as well as urinary calcium and cyclic adenosine-3',5'-monophosphate (cAMP) excretion were significantly increased in group 1. In comparison to patients without therapy, anticonvulsant-treated children showed significantly decreased levels of serum calcium (Ca), ionised Ca (Ca2+), 25-hydroxy vitamin D3 and urinary phosphate (PO4) excretion, and elevated levels of Al-p, iPTH and calcitonin (iCT). It is suggested that limited physical activity results in a mild hyperparathyroid state, which is aggravated in patients on anticonvulsant treatment.

Adolescent↗

Emotionally handicapped youth in transition: issues and principles for program development.

Seriously emotionally handicapped adolescents face significant transition issues as they move through our system of care and attempt to assume adult role responsibilities in the community. Service delivery efforts must be sensitive to these issues and begin to realistically address the transition needs of this population. Nine integrative principles are discussed which appear essential when developing transition services.

Adolescent↗

Impairment, disability, and handicap in multiple sclerosis. A cross-sectional study in an incident cohort in Møre and Romsdal County, Norway.

We conducted a cross-sectional, geographically based study of functional status in an incident cohort of 124 multiple sclerosis (MS) patients with onset of disease from 1 January 1976 to 31 December 1986 in Møre and Romsdal County, Norway. The cohort comprised 58 men (46.8%) and 66 women (53.2%). One hundred and thirteen patients (91.1%) had a primary remitting course of disease and 11 (8.9%) had primary progressive MS. The mean age of onset was 33.3 years (range 14-64), and the mean duration of disease 7.8 years (range 1-23). The Minimal Record of Disability (MRD) of multiple sclerosis was applied to measure the degree of impairment, disability and handicap. The mean Kurtzke Expanded Disability Status Scale (EDSS) score was 3.76 (0-10), and the frequency distribution of the EDSS scores in the cohort was bimodal. Twenty-eight (22.6%) patients in the cohort had marked paraparesis, paraplegia of quadriplegia. Nineteen patients (15.3%) had frequent urinary incontinence, need for almost constant catheterization, or need for constant use of measures to evacuate stools. Five patients (4%) had a severe decrease in mentation or dementia. Forty-eight patients (38.7%) reported frequent problems with fatigue or fatigability preventing sustained physical function. Less than half of the cohort (42.7%) was working full-time, and 49.2% of the patients reported that they received external support to maintain their usual financial standard. Lack of ability to work full-time and accordingly the dependence upon external financial support were significantly associated with the primary progressive course of disease. Fatigue was statistically significantly related to lack of working ability. The estimated cost of MS in the county in 1991 was 48.2 million NOK (approximately 7.531.250 US $) based on the cost-of-illness methodology.

Adult↗

A component analysis of attentional problems of educationally handicapped boys.

Seventy-three educationally handicapped (EH) and 78 regular class, normally achieving (NA) boys grades 3-8 were tested with a series of measures selected to test three components of attention: coming to attention, decision making, and maintaining attention over time. EH and NA samples were subdivided into three gropus by grade level (grades 3-4, 5-6, and 7-8). Based on a teacher-completed behavioral check list, the EH group was further subdivided according to pupils perceived by teachers as hyperactive or nonhyperactive. With the exception of the youngest group, EH and NA samples did not differ from each other on CA, but regular class boys had slightly higher IQs and better reading scores than did their EH peers; EH hyperactives and EH nonhyperactives differed significantly on reading scores, the difference favoring the nonhyperactives. All pupils were individually administered the Children's Embedded Figures Test (CEFT), the Matching Familiar Figures Test (MFFT), and the Children's Checking Task (CCT), the last designed specifically to assess ability to maintain attention over time. EH pupils did not function as efficiently or as accurately on the attentional tasks as did their normally achieving age peers. Significant differences between EH and NA samples were found for CEFT and MFFT errors, as well as for CCT errors of omission and commission. Analyses of the EH group according to hyperactive--nonhyperactive status were for the most part nonsignificant. Correlational analyses yielded low but statistically significant relationships among the attentional measures, but nonsignificant relationships between IQ and the attentional test scores. Findings were consistent with the interpretation that the three hypothesized components of attention are partially independent and thus may have differential influence on pupils performance in school.

Attention↗

A comparison between the handicaps behaviour and skills schedule and the psychoeducational profile.

Comparisons were made of developmental scores (administered with the Psychoeducational Profile [PEP] and the Handicaps Behaviour and Skills Schedule [HBS] for a group of 72 children ages 23 to 148 months. All children had been referred to the Centre of Autism in Leiden, the Netherlands. This Centre is a collaboration between the University clinic of special education and the regional health service. Forty-five children were diagnosed as autistic and 27 as nonautistic but suffering from another disorder. In this study, the correlation between the two instruments is higher than expected, in particular for the group of autistic children (.83). The internal consistency of the subscales of the PEP and the HBS are overall very satisfactory. The Cronbach's alphas of the PEP scales vary from .79 to .96 for the total group and from .77 to .95 for the autistic group. The alphas for the HBS vary from .74 to .92 for the total group and from .20 to .87 for the autistic group, there is one alpha at .20, the rest are .60 or higher.

Autistic Disorder↗

Increasing social interactions of severely handicapped autistic children.

A peer-initiation training procedure was implemented across multiple peer trainers to investigate social interactions between severely withdrawn autistic children and their nonhandicapped peers. For one subject, substantial increases in spontaneous interactions with training and nontraining peers occurred after the peer-initiation procedure was applied across two training exemplars. Spontaneous social interactions continued even after the training procedure was removed. Although experimental control was established with the second subject during training, spontaneous interactions during nontraining periods were primarily with training peers. The results contribute to an emerging data base on the social interactions of autistic and severely withdrawn handicapped children and on peer-initiation training procedures.

Autistic Disorder↗

Increasing simple toy play in profoundly mentally handicapped children: II. Designing special toys.

The purpose of this study was to increase simple toy play in profoundly mentally handicapped children by presenting them with specially designed toys. Twenty children (mean chronological age 14 years, mean mental age less than 1 year) were observed while playing with the special toys, which emitted stimuli (vibration, light, or sound) when appropriately operated. They were also observed with the toys when the stimuli were unavailable. There were large individual differences, but, in general, children interacted significantly more with the experimental toys than with the control toys and engaged in significantly less stereotyped behavior when the experimental toys were available. The implications for clinical and research work are discussed.

Adolescent↗

Increasing simple toy play in profoundly mentally handicapped children. I. Training to play.

The purpose of this study was to increase simple toy play in profoundly mentally handicapped children. Twenty children (mean chronological age, 14 years; mean mental age, less than 1 year) were trained to play with ordinary toys, half by a behavior modification technique and half by a control procedure. The operant training led to some increases in total toy contact but did not appear to be significantly more effective than the control technique in promoting independent constructive play. The possible reasons for the relative lack of success of the behavioral training are discussed.

Adolescent↗

Competency-based training for severely behaviorally handicapped children and their parents.

This paper describes the major components of a treatment program for severely behaviorally handicapped children. The program's goal is to help the children develop the necessary skills to function in regular classrooms or special education classes. The article presents descriptions of the procedures used in the Day School Learning and Treatment Center and the Parent Training Program at the Judevine Center for Autistic Children. Criteria for acceptance, assessment systems, training techniques, and methods for follow-up are outlined. Also, the paper delineates what are considered to have been five major trends in the development of the program.

Adolescent↗

Prevalence of anti-HCV in institutionalised mentally handicapped subjects.

Data on the prevalence of antibody to hepatitis C virus among 278 subjects in an institution for the mentally handicapped were analysed by risk factors. A prevalence of 4% was found, higher than blood donors belonging to the same area (1.4%). No differences in prevalence with regard the length of residence, age, sex, degree of retardation or for presence of HBV markers were observed.

Adolescent↗

Association of increased numbers of peripheral blood double-negative T-lymphocytes with elevated serum IgG levels in severely handicapped children.

CD3+4-8- double negative cells in peripheral blood lymphocytes were examined in 21 severely handicapped children divided into two groups according to serum IgG level. All children were bedridden and were taking multiple anticonvulsants and there were no apparent clinical differences between these two groups. Serum levels of IgG correlated well with percentages of CD3+4-8- double negative lymphocytes in patients of both groups. In comparisons between the two groups, the high IgG group had higher counts of CD3+4-8- double negative lymphocytes in peripheral blood than the normal IgG group. Two distinct types of double negative cells were identified in the patients with high IgG: one had T-cell antigen receptors of gamma delta heterodimers, the other had receptors of alpha beta chains on their surface. As double negative T-cells are reported to have an important role in defence against bacterial infections, the increased numbers of CD3+4-8- T-cells of both phenotypes in the high IgG patients may reflect exposure to repetitive bacterial stimuli or persistent subclinical infection which in many cases, may be undetectable clinically. Moreover, the hyperimmune states shown by the high serum IgG of these patients may result from the appearance of these unique lymphocytes because they are reported to have a helper function for IgG synthesis in vitro. Taken together, the increased numbers of double negative cells in patients with high IgG may reflect activated defence mechanisms and the development of hyperimmune status.

Adolescent↗