bluuebunny/crossref_metadata_2025 huggingface dataset:
Mental outcome in West syndrome: prognostic value of some clinical factors
Authors: FAVATÀ I. , LEUZZI V. , CURATOLO P.
Published: July 2010

ABSTRACT. Fifty‐eight cases of West syndrome (eight idiopathic, 50 secondary) have been followed‐up for a mean period of 5 years 5 months. The relationship has been studied between intellectual status at the final follow‐up and the following variables: etiology, age of onset, neurological examination and developmental assessment at the onset, CT scan findings, short‐term effect of ACTH treatment, and age of acquiring four developmental milestones (sitting unsupported, walking unsupported, uttering the first specific word and using a sentence of two words).

Developmental and/or neurological abnormalities before the onset of the spasms, symptomatic etiology and abnormal CT findings are associated with a low IQ at the final follow‐up.

The age for sitting unsupported and for walking unsupported has proved to be not predictive of intellectual outcome, but the speech development data have shown a highly significant relationship with intellectual development.

Early identification of the children with mental retardation has turned out to be superior to prediction of normal mental outcome.
Microcephaly: a review of genetic implications in its causation
Authors: COWIE V. A.
Published: July 2010

ABSTRACT. Microcephaly is a clinical sign rather than a nosological entity. It may even represent the extreme of normal variation. In pathological cases, it is always caused by an interruption of the neurobiologic processes of induction and cellular migration, or by a catastrophic insult to the central nervous system. The prime cause of this may be environmental or genetic. There is strong evidence for genetic heterogeneity, even among cases of ‘true’ or ‘primary’ microcephaly. Various taxonomies for the classification of microcephaly are discussed, taking into account environmental causation and various genetic mechanisms.
Down's syndrome: intellectual and behavioural functioning during adulthood
Authors: FENNER M. E. , HEWITT K. E. , TORPY D. M.
Published: July 2010

ABSTRACT. Previous cross–sectional studies of Down's syndrome have suggested thatdeficits in cognitive and neurological functioning after the age of 35 years are symptomatic of Alzheimer's disease. It has been claimed that this pattern corresponds to post‐mortem neuropathological findings of Alzheimer's disease in all Down's syndrome patients who die aged over 35 years. In the present study of Down's syndrome patients aged between 19 and 49 years, results showed that, for those over 35 years, intellectual deterioration had occurred in less than a third. No relationship was found between chronological age and the level of self care skills or the presence of disturbed behaviour. Results are discussed in terms of the interpretation of the existing neuropathological literature, the methodology of future studies and clinicaldecisions regarding hospitalized patients with Down's syndrome.
Maternal support for the Down's syndrome stereotype: the effect of direct experience of the condition
Authors: RODGERS C.
Published: July 2010

ABSTRACT. Maternal agreement with the Down's syndrome stereotype (as outlined in the literature) was investigated as a function of direct experience of the condition. Using an adjective checklist devised for this project, mothers of children aged between 3 and 9 years were asked to describe Down's children. Statistical analysis of the data collected revealed that familiarity with the condition resulted in a broader general stereotype (represented by the number of adjectives endorsed). Specifically, mothers of Down's Syndrome children attributed more personality traits to the Down's child than mothers without direct experience of the condition. Mothers of such children also claimed a wider range of personality characteristics for their own child than for Down's children in general. Implications for future research were discussed, as was the value of ‘normalization’ and increased contact with normal peers in the education and integration into the community of Down's children.
Comparison of the daily lives of four young adults who are mentally retarded
Authors: RAYNES N. V. , JOHNSON M. , SUMPTON R. C. , THORP D.
Published: July 2010

ABSTRACT. Least restrictive environments are assumed to be ones in which high‐quality care is to he found. The quality of care provided for four adult males living in hospitals and hostels is described. The data indicate that there are not only differences in the quality of care provided between hospitals and hostels, but variation within both types of setting. Attention is drawn particularly to the differences in care provided within the hostel settings. The use of simple monitoring devices to assist in better practices is recommended.
Cognitive psychology and mental handicap
Authors: O'CONNOR N.
Published: July 2010

ABSTRACT. The psychology of mental handicap has been influenced by two traditions in general psychology, pychometric measurement following Binet and experimental psychology first initiated by Wundt. The weakness of the former is its lack of explanatory power and, of the latter, its lack of sound alignment with the neuropathology of mental handicap. A closer association of experimental psychology and neuropathology might help us to reframe our concepts of intelligence, cognitive functions and their handicaps.
Epidemiological issues in mental retardation
Authors: FRYERS T.
Published: July 2010

The basic epidemiological issues have not changed fundamentally in 30 years but emphases are different. Clarity of concept and definition is essential; we need several definitions for different purposes, and should distinguish global criteria defining groups in some way specifically ‘retarded’ and partial criteria defining groups not exclusive to retardation. Of global definitions, we can distinguish Intellectual Impairment based on IQ, Learning Disability based on educational criteria, and Mental Handicap or Retardation based on service or administrative criteria. The first and second may be co‐terminous for children. The first and third are usually co‐terminous below a certain IQ level, conventionally 50, as SII and SMH/R. This is not so for higher ability groups: Mil and MMR are conceived and defined differently, and suit different research purposes. There may be much unknown need because there have been so few studies of total IQ defined groups above 50. Although organic and psychological factors are very important, the study of MMR needs to recognize its primarily social nature, reflecting determinants of selection into MMR status in legal, organizational and professional structures, activities and attitudes characteristic of particular communities and cultures. If the primary focus is on aetiological factors, natural history, and preventive possibilities, study group should be aetiologically defined and preferably not limited to MR. The same applies to specific impairments, disabilities, diseases, behaviours and disadvantages. The services are also susceptible to epidemiological approaches, descriptive, analytic, interventionist and evaluative, but little is yet available on agency structure and function, professional activities, attitudes and training, legal contexts, and financial constraints. Rigorous outcome studies would greatly benefit rapidly developing services. We know quite a lot about the distribution and associations of SII/SMR, though more is needed, especially to clarify issues of social class distribution. There is a dearth of good research in Mil or MMR, and several large scale new studies could be justified. Work on the epidemiology of aetiological entities has progressed, though there are many gaps. Because of their rarity, we should be looking to bring together all the data on aetiological diagnosis now accumulating in good paediatric units throughout the UK.
Rett syndrome: a survey of North American patients
Authors: COLEMAN M. , BRUBAKER J. , HUNTER K. , SMITH G.
Published: July 2010

ABSTRACT. Parents of 63 North American girls with Rett syndrome filled out retrospective questionnaires in a project of the International Rett Syndrome Association, a parent group. No consistent pattern was revealed of possible etiological factors related to environmental insults; however, additional information gathered supported a genetic etiology. The survey included one pair of identical twins and one child with consanguineous parents. There were 46 male sibs and 34 female sibs. These data weigh against any theory relating etiology to a gene on the X chromosome which is lethal to males in utero. Figures were gathered on a number of clinical items. These were: onset of symptoms between 6 and 18 months of age, 83%; autistic withdrawal, 73%; never walked independently, 23%; hyperventilators with abdominal swelling, 63%; night laughter, 83%. The average age of walking of those who walked was 19 months and the average onset of seizure disorders was between 31/2 and 4 years of age. This paper ends by discussing the limitations of a parent questionnaire.
Criterion‐related versus non‐criterion‐related prompt training with severely mentally handicapped children
Authors: STRAND S. C. , MORRIS R. C.
Published: July 2010

ABSTRACT. Thirty‐three severely mentally handicapped children were involved in a study to evaluate the effectiveness of three training programmes in teaching visual discriminations. Three matched sets of subjects each received both a size and an intensity discrimination with either: (1) size prompting; (2) intensity prompting; or (3) no prompting (trial‐and‐error training). The prompted groups therefore received both criterion‐related (CR) and non‐criterion‐related (NCR) training. Order of presentation of the discriminations was counterbalanced across subjects. There was a significant main effect of training group with both size and intensity prompting groups making significantly fewer errors than the trial‐and‐error group (P<0.05 in both cases), bui not differing significantly from each other. Significant linear trends for increasing errors across CR, NCR and trial‐and‐error training programmes were found for both the size discrimination (PP<0.01), and intensity discrimination (PP<0.05). More children acquired the discriminations when trained with a CR prompt than with a NCR prompt or trial‐and‐error procedure, and all four children who failed lo acquire discriminations with NCR prompts subsequently acquired them when trained with a CR programme. The results arc discussed in relation to cue discriminability and theoretical explanations involving overshadowing are considered.
A computer‐aided programme for low‐functioning persons: a reply to Odor and Aitken
Authors: LANCIONI G. E. , OLIVA D.
Published: July 2010

ABSTRACT. The present paper is a reply to the comments of Odor (1988) and Aitken (1988) on the present authors’ study concerning a computer‐aided programme for low‐functioning persons (Lancioni & Oliva, 1988). The research problem and the objectives of the study will be restated first. Subsequently, a series of issues on which Odor and Aitken have commented will be addressed. These issues include the activities presented to the subjects, the characteristics of the computer system, the differences between computer and card systems, intrinsic motivation and external reinforcement, the kind of subjects who can benefit from the computer system, and ethical aspects.
Semantic encoding by mildly retarded and non‐retarded individuals
Authors: STAN E. A. , MOSLEY J. L.
Published: July 2010

ABSTRACT. Three comparable groups of mildly retarded inviduals were required to encode words according to one of the levels of processing paradigm, a distinctiveness of encoding paradigm or a control condition in which encoding was not influenced in any manner. Their performance on an immediate but unexpected recognition task was compared to that of three comparable groups of equal‐MA and three comparable groups of equal‐CA individuals. The equal‐CA group was the only group advantaged by both the levels of processing (LOP) and the distinctiveness of encoding (DOE) manipulations. The solid performance of the equal‐MA subjects in the control, the LOP and the DOE conditions was attributed to the efficient use of episodic memory. The poor performance of the mildly retarded subjects across these conditions was attributed to the lack of semantic organization in permanent memory and to an inefficiency in the use of episodic memory.
Psychometric properties of the Social Training Achievement Record (the STAR profile)
Authors: STURMEY P. , CRISP A. G. , NEWTON T. J.
Published: July 2010

ABSTRACT. Seven hundred and sixty‐eight Social Training Achievement Records were collected independently in two settings during routine assessment of mentally handicapped people, mostly adults in hospital. All scales and total scores were highly internally consistent. Factor analysis on one sample yielded two factors named General Adaptive Behaviour and Community and Equipment Skills. This factor structure was replicated on the second sample. This factor solution is highly similar to that reported for the Adaptive Behavior Scale (Nihira, 1976). Two new scales were derived on the basis of these factor analyses which were also highly internally consistent. Parallel forms of these scales were also satisfactorily developed. Total scores and scores on the two new scales were adequate in routine clinical practice and showed good concurrent and criterion group validities. Reliabilities of individual items were less satisfactory. Suggestions for future developments of this scale are made.
The development of Alzheimer's disease in Down's syndrome assessed by auditory event‐related potentials
Authors: BLACKWOOD D. H. R. , ST CLAIR D. M. , MUIR W. J. , OLIVER C. J. , DICKENS P.
Published: July 2010

ABSTRACT. Several studies have reported changes in auditory event‐related potentials in patients with Alzheimer's type dementia, These include an increase in latency and a reduction in amplitude of the P300 (P3) response, a late positive component generated about 300 ms after an unexpected stimulus. Alzheimer's type dementia is an almost invariable acompaniment of ageing in Down's syndrome. This study was designed to assess the usefulness of the auditory P300 response as a measure of the onset of dementia in Down's subjects, who because of poor language development may be difficult to assess by psychological tests. Auditory event‐related potentials were recorded from 89 Down's subjects, aged 16–66 years. A control group of 29 mentally retarded subjects with fragile‐X syndrome and 83 normal volunteer controls were also tesled. Clinical psychological testing found evidence of dementia in 16 Down's subjects and none with fragile‐X. Furthermore, in the Down's population but not the fragile‐X or control groups, there was a marked increase in P300 latency with age starting around 37 years. In controls, the effect of age on P300 latency became significant some 17 years later around the age of 54 years. The premature effect of age on P300 in Down's syndrome was due to the prolonged P300 latency in the 16 subjects showing signs of dementia. It was confirmed that P300 latency increase reflects the development of Alzheimer's dementia in Down's subjects.
Sensorimotor intelligence in severely mentally handicapped children*
Authors: MACPHERSON F. , BUTTERWORTH G.
Published: July 2010

ABSTRACT. The pattern of cognitive development in severely mentally handicapped children was assessed with the Uzgiris‐Hunt (1975) Scales of Infant Psychological Development, which measure pre‐verbal sensorimotor intelligence. The normative data on which Uzgiris & Hunt constructed the Scales were used for the comparison. The results indicated considerable deficits in the handicapped children, particularly in imitation.
The effects of stereotyped behaviour on orienting and tonic cardiac activity
Authors: MacLEAN W. E. , LEWIS M. H. , FIDLER P. S. , BRYSON‐BROCKMANN W. A. , BAUMEISTER A. A.
Published: July 2010

ABSTRACT. The relationship of cardiac activity to stereotyped behaviour was studied in profoundly mentally retarded persons, using both intra‐ and inter‐group comparisons. The first intra‐group comparison involved examining the relative magnitude of an orienting response to an auditory stimulus during the occurrence or nonoccurrence of stereotyped body rocking. While no differences were found between periods of rocking and non‐rocking when behavioural measures of orienting were used, smaller changes in post‐tone heart rate were observed during periods of stereotyped rocking. A second intra‐group comparison found that stereotyped behaviour was associated with a significant increase in heart rate but no change in heart rate variability when compared to periods of no stereotyped activity. Finally, using an inter‐group comparison, individuals who displayed stereotyped body rocking exhibited higher tonic heart rates and lower heart rate variability during periods of no stereotyped behaviour than was observed in matched control subjects. This result suggests that stereotyped behaviour may be correlated with reduced vagal tone.
Prevalence of dyskinesia and related movement disorders in a developmentally disabled population*
Authors: STONE R. K. , MAY J. E. , ALVAREZ W. F. , ELLMAN G.
Published: July 2010

ABSTRACT. The prevalences and inter‐relationships of five types of movement disorders were evaluated in a large, developmentally disabled (DD) population (n=1227); prevalence was evaluated with regard to severity, age, gender and antipsychotic‐drug (APD) exposure. Dyskinesia was found in 48% of the sample, dystonia in 29%, akathisia in 13%, Parkinsonism in 3% and paroxysms in 4%. Many persons had more than one symptom so that 72% had one or more of the five target symptoms. Although the five movement‐disorder categories were not mutually exclusive, analysis supported the individuality of the categories as defined in this study. The prevalences of dyskinesia and Parkinsonism were considerably greater than those in the general population. On the other hand, the prevalence of dyskinesia was similar to that reported for psychiatric and institutionalized geriatric populations. Parkinsonism increased with age and male gender, while dyskinesia increased with age and female gender. APD‐exposure was significantly correlated only with akathisia.
A method of displaying assessment information about people with multiple handicaps as a disability profile
Authors: ROHDE J. , FARMER R. , GLOVER G. , NURSEY A.
Published: July 2010

ABSTRACT. A method is described of displaying information about the range of multiple disabilities experienced by individuals who are also mentally handicapped, using the assesments available on a Mental Handicap Register. The presentation, in the graphical form suggested, enables groups to be compared whilst retaining details of an individual's problems.
A case of Down's syndrome, insulinoma and anorexia
Authors: MORGAN J. R.
Published: July 2010

ABSTRACT. The report describes a case of a 35‐year‐old female with Down's syndrome, insulinoma and anorexia. It is believed to be the first time such an association has been reported. Diagnostic and management problems are discussed with particular reference to people with a mental handicap.
Basal metabolic rate in healthy Down's syndrome adults
Authors: SCHAPIRO M. B. , RAPOPORT S. I.
Published: July 2010

ABSTRACT. Basal metabolic rale (BMR) was measured on 2 consecutive Jays with an open‐circuit, indirect calorimeter in 16 healthy subjects with trisomy 21 Down's syndrome (DS) aged 22‐35 years and 11 healthy control volunteers aged 20‐31 years. BMR measurements Jut mil differ on consecutive days in either the DS or control groups. Absolute BMR, BMR corrected for surface area, and HMR corrected tor lean body mass did not differ between the DS subjects and the volunteers. The per cent of predicted BMR also did not Jitter between the two groups. These results suggest that systems controlling basal metabolism are not affected by extra gcnomic material from chromosome 21.
Trader Willi syndrome with hypothyroidism
Authors: BHATE M. S. , ROBERTSON P. E. , DAVISON E. V. , BRUMMITT J. A.
Published: July 2010

ABSTRACT. A case of Prader Willi Syndrome who suffered from hypothyroidism is described. This patient on cytogenetic examination was found to have Mosaic 46,XX/46,XX,det(15Kq11.1q11.2) karyotype.
The Miller‐Dicker syndrome: a case report and review of the literature
Authors: IZMETH M. G. A. , PARAMESHWAR E.
Published: July 2010

ABSTRACT. A case of Miller‐Dieker syndrome is reported. A 6‐month‐old baby girl has the characteristics of the syndrome. Post natal growth deficiency: microcephaly, profound mental retardation. Craniofacial defects: micrognathia, ear abnormalities, anteverted nares, wrinkling of forehead, bitemporal growing of high forehead. Neurological abnormalities: opisthotonos, epilepsy, initial hypotonia. The literature is reviewed.
Urinary levels of taurine in mentally retarded children
Authors: NALINI K. , AROOR A. R. , RAO A.
Published: July 2010

ABSTRACT. Urinary taurine levels were estimated in 29 mentally retarded children. These levels were then compared with those of normal healthy children. The urinary taurine levels were significantly higher in the mentally retarded subjects. There is probably an intriguing relationship between taurine levels and mental retardation.
Occupational engagement of low‐functioning individuals: extending the applicability of a computer‐aided programme
Authors: LANCIONI G. E. , OLIVA D. , RAIMONDI D. , CIATTAGLIA F.
Published: July 2010

ABSTRACT. The present study addressed two questions concerning a computer‐aided programme for supervising occupational activities for low‐functioning individuals. The first question was about the possibility of using the programme for extended time periods. The second question concerned the viability of the programme for subjects who require physical prompting in addition to normal supervision (e.g. instructions and reinforcement). Three adolescents participated in the study. Two of them (who could perform under normal supervision) were selected for investigating the first question. The other subject (who required caretaker prompting) served for the second question. The results for the first two subjects showed that the computer‐aided programme could easily ensure engagement in constructive activities for periods of about 30 min. The data for the third subject showed the establishment of moderate responding. The practical implications of the findings are discussed.
Akathisia in neuroleptic medicated mentally handicapped subjects
Authors: GANESH S. , RAO J. MURTI , COWIE V. A.
Published: July 2010

ABSTRACT. Sixty‐six neuroleptic medicated mentally handicapped subjects in a hospital were studied to determine the prevalence of drug‐induced akathisia. Tardive dyskinesia was also rated on the AIMS scale. Only motor manifestations of akathisia could be assessed as the subjective component of akathisia was difficult to elicit in this population with difficulties in verbal communication. As all the subjects had been on neuroleptics for at least 3 years, only chronic akathisia could be studied. Five subjects had akathisia. Correlational analysis did not reveal any specific associations with any of the demographic, clinical and pharmacological variables studied. A step‐wise multiple regression analysis indicated that younger age could be a predictor. Tardive dyskinesia was associated in two of the subjects. The overall conclusion was that 7% of the subjects had chronic akathisia and no specific risk factors could be identified.
Polycystic ovary disease, manic‐depressive illness and mental retardation
Authors: GHAZIUDDIN M.
Published: July 2010

ABSTRACT. Presence of polycystic ovary disease is described in a mentally retarded woman who later developed features of manic‐depressive illness. The possibility that the three conditions might interrelate pathophysiologically is discussed.
Assessing levels of state and arousal in individuals with profound handicaps: a research integration
Authors: STERNBERG L. , RICHARDS S.
Published: July 2010

ABSTRACT. To determine optimal learning conditions in the profoundly handicapped, an approach which assesses state through a prediction analysis is proposed.
Neuro‐developmental study of profoundly mentally handicapped children in hospital care
Authors: MINNS R. A. , WONG B. , BROWN J. K. , ERASER W. I.
Published: July 2010

ABSTRACT. Swedish estimates of the need for long‐term intensive health care provision for profoundly mentally handicapped children were compared with an Edinburgh survey, and the neurodevelopment and health characteristics of Edinburgh children with profound mental handicap who required prolonged admission are described. The extent of their disabilities indicates the complexity of the task of designing alternatives to their present hospital provision which will adequately meet their health needs.
School achievement in treated PKU children
Authors: FISHLER K. , AZEN C. G. , FRIEDMAN E. G. , KOCH R.
Published: July 2010

ABSTRACT. Phenylketonuric children who were treated early and maintained a phe‐ restricted diet through age 10 were compared with those who discontinued the diet after age 6 on a standardized test of intelligence, school achievement, language and perceptual skills. Mean IQ, reading and spelling test scores improved between ages 6 and 10 for the on‐diet children in comparison to those who were off‐diet. However, mean scores on arithmetic, language and perceptual skills declined at a uniform rate for both groups. Children with PKU scored significantly lower than did their non‐PKU siblings on tests of visual perception and visual‐motor skills. Because the school years, particularly, the early teens, pose increased stress in affected PKU individuals, it is recommended that dietary restriction at least through the high school years is prudent for their optimal physical, mental, emotional and educational growth.
Atlanto‐occipital instability in Down's syndrome
Authors: COLLACOTT R. A. , ELLISON D. , HARPER W. , NEWLAND C. , RAY‐CHAUDHURT K.
Published: July 2010

ABSTRACT. A patient with Down's syndrome and asymptomatic atlanto‐occipital instability is described. The relationship between atlanto‐occipital instability, atlanto‐axial instability and generalized ligamentous laxity, is discussed.
Trisomy 12p mosaicism syndrome
Authors: KARKI C. B. , WALTERS R. M.
Published: July 2010

ABSTRACT. A case of trisomy 12p mosaicism originating de novo in a 21‐year‐old profoundly mentally handicapped female who presented with severe behaviour problems is described. The 12p syndrome has been confirmed by clinical, cytogenetic and biochemical studies. The findings are compared with those of other cases with trisomy 12p arising de novo.
The effects of music therapy on a group of profoundly mentally handicapped adults
Authors: OLDFIELD A. , ADAMS M.
Published: July 2010

ABSTRACT. An investigation of the effects of music therapy on profoundly mentally handicapped adults is reported. The therapy was directed at achieving specific objectives for individuals and the study compared the efficacy of music therapy and play activities in achieving these objectives. Four subjects out of the 12 included in the therapy groups were studied intensively. All subjects received both treatments in a reversal design with 20 weekly sessions of each treatment. Videotapes of the treatment sessions were analysed using a time‐sampling method to record the occurrence of behaviour indicating the achievement of the individual treatment objectives. Staff behaviour in the treatment sessions was also observed to investigate the role of staff attention in mediating the treatment effects. The observations of staff behaviour showed that, overall, there was less individual attention given during music therapy than in play sessions, that there was a much greater role differentation among the staff present in the music therapy groups and that individual clients in the groups received markedly unequal amounts of staff attention. For all four subjects studied in detail, some differences in behaviour were shown between the treatments and most, but not all, of these differences indicated greater effectiveness of music therapy. For one subject showing higher performance in play sessions than in music therapy, this could be attributed to a higher level of staff attention for this subject. The differences in favour of music therapy could not be accounted for in this way. It is concluded that music therapy is a useful treatment approach with profoundly handicapped people.
Free recall of lists of word and sign labels by severely handicapped children
Authors: BOWLER D. M. , KIERNAN C.
Published: July 2010

ABSTRACT. Two experiments are reported which tested short‐term recall of lists of four sign or four word labels by severely handicapped children. The findings suggest that the subjects were more efficient at processing words than signs in short‐term memory, and that their organization of sign and word lists were affected by the length of time the material had to be held in short‐term store. Both of these effects were influenced by whether or not the subjects had to use the stimuli as labels when making a response.
Comprehension of syntactically indicated sequence by Down's syndrome and other mentally retarded adults
Authors: KERNAN K. T.
Published: July 2010

ABSTRACT. The ability to comprehend syntactically ordered sequence by Down's syndrome adults and adults with mental retardation of no specific aetiology was studied. Both samples exhibited patterns of comprehension that resembled those of children who are not mentally retarded. However, the Down's syndrome individuals had significantly more difficulty when sequence was indicated by syntax with no supporting extralinguistic context. The Down's syndrome adults appeared to be arrested at an earlier stage of development than the other mentally retarded adults.
Dietary increase in serotonin reduces self‐injurious behaviour in a Down's syndrome adult
Authors: GEDYE A.
Published: July 2010

ABSTRACT. A 26‐year‐old profoundly mentally handicapped woman with Down's syndrome and severe self‐injurious behaviour was treated by dietary means. Treatment consisted of regular institutional entrees, supplemented with serotonin‐containing foods, and modified to ensure dietary sources of potassium each intake and low protein relative to carbohydrate. Dramatic benefit was evident the first day and, after 6 months self‐hitting was still close to 90% lower than baseline rates. Relevant literature was discussed regarding why a serotonergic approach was chosen for reducing self‐injury.
Autism, profound mental retardation and atypical bipolar disorder in a 33‐year‐old female with a deletion of 15q12
Authors: KERBESHIAN J. , BURD L. , RANDALL T. , MARTSOLF J. , JALAL S.
Published: July 2010

ABSTRACT. A case report of a 33‐year‐old woman wich atypical bipolar disorder, autistic disorder, profound mental retardation and a chromosomal anomaly is presented. This patient is behaviourally similar to a patient reported by Akuffo et al. (1986), who apparently did not have a chromosomal investigation. The chromosomal anomaly and similarities between the two patients are discussed.
Vitamin A gastrointestinal absorption in persons with Down's syndrome
Authors: PUESCHEL S. M. , HILLEMEIER C. , CALDWELL M. , SENFT K. , MEVS C. , PEZZULLO J. C.
Published: July 2010

ABSTRACT. The total daily vitamin A intake, physical signs associated with vitamin A deficiency, and the response to a vitamin A challenge were studied in 33 patients with Down's syndrome and in 14 intellectually normal persons (comparison group). The evaluation of detailed dietary histories revealed that the study subjects' mean daily vitamin A intake was similar to the recommended daily allowance for adults and that there was no significant difference of the mean daily vitamin A intake between study and comparison groups (5029 IU and 5706 IU, respectively). Subjects with Down's syndrome had more symptoms usually seen in patients with hypovitaminosis A than the persons in the comparison group. The baseline serum vitamin A levels in the Down's syndrome and comparison groups were within the normal range (106.0 μg/dl and 136.5 μg/dl, respectively). The vitamin A absorption curve of persons with Down's syndrome paralleled that of normal individuals and no significant difference of vitamin A levels between study and comparison groups was observed except for the 6‐h values. Also, the absorption differential ( ‐0‐h value) did not show a significant difference between the two groups. Thus, these investigations do not support previous reports of significantly decreased vitamin A absorption in individuals with Down's syndrome.
Prevalence of stuttering and cluttering in adults with mental handicaps
Authors: STANSFIELD J.
Published: July 2010

ABSTRACT. A survey (involving questionnaire, screening and assessment procedures) of 793 adults with mental handicaps revealed that 6.3% of the population had ideopathic dysfluencies. Issues in the differential diagnosis between stuttering, cluttering and other dysfluency are considered, and the contaminating factors involved in this diagnosis discussed.
Antiepileptic polypharmacy, psychosocial behaviour and locus of control orientation among mentally handicapped adults living in the community
Authors: ESPIE C. A. , GILLIES J. B. , MONTGOMERY J. M.
Published: July 2010

ABSTRACT. The relationship beteween psychosocial behaviour, antiepileptic regime (polytherapy compared to monotherapy) and seizure frequency was explored in a sample of mentally handicapped adults living in the community. Locus of control perceptions were studied in relation to seizure frequency. Antiepileptic polypharmacy was found to have a detrimental effect upon psychosocial functioning independent of seizure frequency, which is itself a risk factor for social disturbance; and external perceptions of locus of control predominated. The distribution of drugs used is reported. These results are discussed in the context of implications for future research in the areas of coping, mental handicap and antiepileptic pharmacotherapy.
Clinical trial of ‘off diet’ older phenylketonurics with a new phenylalanine‐f ree product*
Authors: CROWLEY C. , KOCH R. , FISHLER K. , WENZ E. , IRELAND J.
Published: July 2010

ABSTRACT. Ten PKU subjects were treated with Product 196 for one year. Product 196 is a special phenylalanine‐free dietary supplement consisting primarily of essential amino acids, carbohydrate, Vitamin C and some minerals, made by Scientific Hospital Supplies Limited, Liverpool, England. It is intended for persons with phenylketonuria who are not consuming a phenylalanine‐restricted diet. During the year that the 10 subjects were on the product, they remained asymptomatic, maintained their weight and appeared healthy. Subjective behavioural improvement was noted in six but no significant changes in intelligence were noted.
Frequency and type of incidents occurring in urban‐based group homes
Authors: SPANGLER P. , GILMAN B. , BORDE R. LA
Published: July 2010

ABSTRACT. A total of 770 persons with mental retardation were evaluated to determine the frequency and types of incidents occurring in urban‐based group homes. All persons resided in group homes located in a large city. Over an 18‐month period, a total of 3075 incidents in 14 categories, were documented. Two categories, behavioural incidents and medication errors, comprised 61% of all incidents recorded. Further, a small number of clients accounted for a disproportionately high number of behavioural incidents. Results point to the need to apportion resources based on frequency and types of incidents occurring in group homes.
Hepatitis B carriers in a centre for the mentally retarded in Finland and immunogenicity of hepatitis B vaccine
Authors: MÖLSÄ P. K.
Published: July 2010

ABSTRACT. The institutionalized population and staff in a centre for the mentally retarded were tested retrospectively, and 1.6% hepatitis Bs antigen and 0.6% hepatitis Be antigen carriers were found among persons with mental retardation. The 40 subjects, residents and staff, with most risk of acquiring hepatitis B were given vaccination. The immunogenicity of the H‐B‐Vax vaccine was followed up prospectively and, in 7.5% of the vaccinated persons, even re‐vaccination gave an unsatisfactory result.
Contribution of ocular examination to the diagnosis of foetal alcohol syndrome in mentally retarded children
Authors: STRÖMLAND K.
Published: July 2010

ABSTRACT. An ophthalmological study was conducted on a series of 28 Swedish children suffering from mild mental retardation without an obvious aetiological diagnosis. The eye examination supported a diagnosis of foetal alcohol syndrome in five out of six suspected cases and revealed two additional not previously suspected (25%), Eye anomalies were observed in 16 patients (57%) with fundus abnormalities (optic nerve hypoplasia, retinal dystrophy) being most frequent, occurring in 11 cases (39%). Four patients had substantially reduced visual acuity in both eyes. Eight children had abnormal features of the face and outer eye region. Ocular motility disturbances (strabismus or nystagmus) were seen in five patients. The ophthalmological study disclosed that at least 10% of this group of children with mild mental retardation suffered from the foetal alcohol syndrome. Il is concluded lhat, in children with mental retardation, an ocular examination is a valuable diagnostic tool for revealing prenatal origin.
Specific motor abilities associated with speech fluency in Down's syndrome
Authors: DEVENNY D. A. , SILVERMAN W. , BALGLEY H. , WALL M. J. , SIDTIS J. J.
Published: July 2010

ABSTRACT. Eight adult male stutterers with Down's syndrome (DS) were compared with a matched group of fluent speakers with DS on verbal and manual motor production tasks at two levels of complexity. The simpler tasks involved diadochokinetic rate (syllable repetition) and finger tapping; the more complex tasks involved the imitation of sentences and placing pegs in the grooved pegboard. On both verbal and manual tasks, stutterers were faster on the simpler but slower on the more complex tasks than were the fluent speakers. The findings suggest that stutterers with DS have a different motor organization than fluent speakers with DS.
Evaluation of a community care project for people with learning difficulties
Authors: FLEMING I. , KROESE B. STENFERT
Published: July 2010

ABSTRACT. The quality of life and adaptive behaviour of a group of 17 adults with learning difficulties who transferred from a long‐stay hospital or hostel into small community‐based group homes was assessed before and 1 year after the move. It was found that the increase in adaptive behaviour was largely dependent on structural staff procedures and there was a bias toward achievement in house‐bound skills. Subjects had a low level of community presence and few meaningful relationships. Implications for service planning are discussed.
Rectal polyposis in tuberous sclerosis
Authors: GOULD S. R. , STEWART J. B. , TEMPLE L. N.
Published: July 2010

ABSTRACT. Microhamartomatous rectal polyps are described in 11 out of 15 patients with tuberous sclerosis (TS). These readily identifiable polyps are of no clinical significance but their prevalence and unique nature rank them as of equal importance to other better known markers of TS such as the facial angioflbromata (adenoma sebaceum).
Offer sequences between parents and young children with Down's syndrome
Authors: WOOTTON A. J.
Published: July 2010

Videorecordings were made in the homes of six children with Down's syndrome whose developmental ages ranged between about 1 and 2 years. Analysis of sequences initiated by parental offers to their children suggests that these children's response behaviour is sensitive to both non‐verbal aspects of the offerer's behaviour and the context in which the offer is made.
The effect of social contact on college students’ attitudes toward severely handicapped students and their educational integration
Authors: BEH‐PAJOOH A.
Published: July 2010

Research on integration suggests that the major benefit of educational integrated programmes is the facilitation of intergroup contact between mentally handicapped and non‐handicapped students. The present study was designed to investigate the effect of different amounts of social contact with severely handicapped students on attitudes and emotional reactions of college students towards such students and their integration into mainstream education. The study was carried out in a tertiary college which has a link programme with a local school for pupils with severe learning difficulties. The results indicated that those participants who had social contact with severely handicapped students held more favourable attitudes and emotional reactions toward such students than did those who had no social contact with this group. The study concludes that the educational integration programmes can positively modify attitudes and emotional reactions of college students towards severely handicapped students. However, in order to implement an effective link or integration programme and to foster attitudes more favourable towards severely handicapped students, the study recommends that more opportunities for intergroup contact and social interaction between non‐handicapped and handicapped students need to be provided.
The sterilization of people with a mental handicap: the views of parents
Authors: BAMBRICK M. , ROBERTS G. E.
Published: July 2010

The issue of sterilization in people with mental handicap has re‐emerged in recent years. Historical aspects are briefly reviewed, and attention is drawn to the different context in which this issue has been revived. A postal survey of parental views was undertaken. Fifty‐three per cent of the respondents indicated that they had or would consider sterilization for their child with mental handicap, and a large majority of the parents felt that they alone, or in conjunction with a doctor, should be able to consent on behalf of the person with mental handicap. Consideration needs to be given to parental anxieties with regard to sexual and reproductive health issues which are surfacing with the current emphasis on community care and the philosophy of normahzation.
The validity and applicability of the ADIECAS classroom rating scale in a sample of children with Down's syndrome
Authors: TURNER S. , SLOPER P. , KNUSSEN C.
Published: July 2010

The ADIECAS classromm behaviour rating scale (Evans & Hogg, 1984) was used with a sample of 123 children with Down's sydrome as part of a wider study of child and family adaptation. According to Evans & Hogg, the ADIECAS scale measures two types of behaviour: attentiveness‐distractability, and inhibition‐excitability. Such measures of behaviour style may be related to behaviour problems and child and family relationships for children with mental handicap. The factor structure of the 16 ADIECAS items was investigated by using confirmatory factor analysis within the LISREL program. Overall, the original factor structure was found to be a poor fit to the new data, in that, while the distractability factor replicated well, the excitability factor split into three. Alter examining the components of the four factors, two were discarded as containing unreliable items, and goodness of fit statistics on the two revised factors, using 10 of the original 16 items, gave the best results. Thus, the factors structure showed only limited replicability with a different subject group from the original Evans & Hogg study.
The performance of children with a moderate mental handicap on simple counting tasks
Authors: McEVOY J. , McCONKEY R.
Published: July 2010

ABSTRACT. This study examines both oral and object counting, and the ability to read numerals in a group of 51 children with a moderate degree of mental handicap. The findings indicate deficiencies in basic counting competencies across a wide spread of ability. There is also evidence for a distinction between oral and object counting on a cognitive skill basics. The findings are discussed within a remediation context.
Factors relating to self‐sufficiency in children with Down's syndrome
Authors: TURNER S. , SLOPER P. , KNUSSEN C. , CUNNINGHAM C.
Published: July 2010

ABSTRACT: An index of self‐sufficiency in children with Down's syndrome was developed for use in a study of the process of adaptation in families living in the Greater Manchester area. The children were 6‐14 years old, and living at home. Index scores were based on the mothers’responses to a questionnaire covering their children's personal and domestic functioning and community self‐sufficiency, and were obtained from 111 of the 117 mothers who took part in the study. A wide range of child‐ and family‐related variables were found to be significantly associated with self‐sufficiency scores on a univariate level. Multiple regression analysis was used to identify those variables which best predicted the level of self‐sufficiency achieved. After the child's mental age, the most significant variables were excitability, behaviour problems, the extent the mother used practical means of coping, and the level of social activity experienced by the child. Together, these five variables explained 63% of the variance in self‐sufficiency scores. The dynamics by which such factors may relate to selfsufficiency in children with Down's syndrome are discussed, and intervention aimed at promoting the use of practical ways of coping by parents is suggested.
Sleep patterns and sleep problems amongst people with mental handicap*
Authors: ESPIE C. A. , TWEEDIE F. M.
Published: July 2010

ABSTRACT. This paper is in two parts. The first part reviews the available literature describing sleep patterns of people with mental handicap and the nature and prevalence of sleep disorders amongst this client group. Both electroencephalographic studies and informant‐based reports are included. The literature is small, particularly in terms of the latter reports, and most of the information from both E.E.G. and informant sources describes populations of children with mental handicap. The need for further investigation of sleep and sleep problems, particularly amongst adults with mental handicap, therefore, becomes evident. The second part presents the results of a detailed sleep survey of 120 adults with mental handicap, approximately half of whom were resident in hospital and half in the community. A descriptive summary of the total sample is provided. Fifteen per cent of the sample presented significant sleep problems, particularly in the form of intermittent wakenings. The results of comparisons between (1) good and poor sleepers (2) hospital and community residents and (3) people with mental handicap and‘normal’adults (using data from another study) are then presented. The relationships between sleep and daytime functioning and the potential usefulness of behavioural approaches to management of sleep pattern are discussed.