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PubMed1993

On the formulation and computer implementation of an age-dependent two-sex demographic model.

A two-sex age-dependent demographic model is formulated within the framework of a stochastic population process, including both time-homogeneous and time-inhomogeneous laws of evolution. An outline of the parametric components of the system, which expedite computer implementation and experimentation, is also given. New features of the model include a component for couple formation, using the class of Farlie-Morgenstern bivariate distributions to accommodate age preferences in selecting marriage partners, a component for couple dissolution due to separation or divorce, and an outline of techniques for initializing a two-sex projection given scanty information. For the case of time-homogeneous laws of evolution, stability properties of two-sex models that are analogs of those for one-sex models are difficult to prove mathematically due to nonlinearities. But computer experiments in this case suggest that these properties continue to hold for two-sex models for such widely used demographic indicators as period crude birth rates, period rates of natural increase, and period age distributions, which converge to constant forms in long-term projections. The values of the stable crude birth rate, rate of natural increase, and quantiles of the stable age distribution differ markedly among projections that differ only in selected values of parameters governing couple formation and dissolution. Such experimental results demonstrate that two-sex models are not merely intellectual curiosities but exist in their own right and lead to insights not attainable in simpler one-sex formulations.

Age Factors↗
PubMed1976

Peptide-binding macromolecules in the blood of seriously ill or mentally retarded patients.

This report describes macromolecules that bind (des-aspartic acid1)-angiotensin II, the des aspartic acid1 derivative of angiotensin I, and several biologically active and inactive analogues of these polypeptides. The macromolecules were found in the plasma of approximately 2 per cent of ambulatory adults and hospitalized children and 32 per cent of the patients at two institutions for the mentally retarded. The binding properties of these macromolecules were studied by incubating with peptides labeled with 125iodine, and separating bound from free labeled peptide using small gel filtration columns. The peptide-binding macromolecules from several patients were compared. They showed very similar specificity for a group of arginyl peptides of the des-aspartyl1-angiotensin sequence. The plasma binders differed from one another in their optimum pH and their mobility in electrophoretic fields. Those with more acid pH optima displayed more rapid electrophoretic mobility. The binders fell into two classes based on apparent molecular weight, approximately 140,000 and 250,000. Those with the higher apparent molecular weight contained a large proportion of binder that could be precipitated with antiserum to human IgA. Kinetic measurements showed that the plasma binders were somewhat heterogeneous with respect to affinity for (des-asp1)-angiotensin, with apparent association constants ranging from 10(7) to 10(8) M-1. Binding activity was labile to heat, and to treatment with pepsin or trypsin. It was inhibited by calcium, protamine, streptomycin, and some other cationic compounds. The plasma peptide binder differed in specificity and molecular weight from soluble angiotensin-binding molecules extracted from tissues, and from properties expected of a receptor for angiotensin. These macromolecules may be useful reagents for measuring (des-asp1)-angiotensins. Their presence in plasma samples may interfere with angiotensin assays in some circumstances.

Angiotensin II↗
PubMed1976

Effects of frequency of photic stimulation upon autistic and retarded children.

The present study was designed to determine if frequency of repetitive photic stimulation could affect the response rate of an operant upon which such stimulation was made contingent. To test this hypothesis, we trained retarded and autistic children, equated for mental age, to pull a lever to obtain photic stimulation of different frequencies. It was found that (a) response rates of autistic children were related to the frequency of stimulation, while retarded children showed no such consistency. (b) The two groups did not differ in the average duration of time they engaged in stereotypies. (c) During a subsequent extinction session, two autistic children ceased responding, but one of two retarded children did not. (d) Noncontingent photic stimulation suppressed a food-motivated operant in one autistic child. These results were interpreted as evidence for frequency-dependent properties of repetitive visual feedback in autistic children. In addition, support was given to the position that performance deficits in autistic children correlating with the occurrence of stereotypies result from competition of different sources of reinforcement.

Age Factors↗
PubMed2005

A measure of subjective burden for dementia care: the Caregiving Difficulty Scale--Intellectual Disability.

BACKGROUND: It has been suggested in the literature on family caregiving for persons with Alzheimer's dementia (AD) that levels of objective and subjective burden among carers often predict institutionalization of the persons with AD. There is a paucity of measures to assess whether perceived burden among formal caregivers may also predict movement to more restrictive settings for persons with intellectual disabilities (ID) and AD. This study focused upon the development of a measure of subjective burden, The Caregiving Difficulty Scale - Intellectual Disability (CDS-ID) as a first step in addressing this measurement deficit. METHODS: An existing caregiver subjective burden scale, the Caregiving Hassles Scale (CHS) was adapted for use with 203 staff caregivers of persons with ID and AD. Preliminary testing of existing CHS items and proposed new items was carried out in two countries, Ireland and the USA. Confirmatory factor analysis with the existing items and exploratory factor analysis with existing and proposed new items for the scale was used to establish the content and test the psychometric properties of a revised scale, the CDS-ID. RESULTS: On the existing CHS items, staff carers appeared to experience greater subjective burden than has been reported for family caregivers. However, the psychometric properties of the CHS found with this population were poor. Factor analysis produced a revised scale, the CDS-ID with three subscales with Cronbach alphas ranging from 0.75 to 0.93 and 38 items overall. CONCLUSIONS: This new scale when used with objective burden and other scales offers an opportunity to more systematically measure the difficulties staff experience when caring for persons with ID who present with symptoms of AD.

Adult↗
PubMed2001

The Psychogeriatric Assessment Scales (PAS): psychometric properties in French and German speaking populations.

OBJECTIVES: To investigate the structure and measurement properties of the Psychogeriatric Assessment Scales (PAS) in a community sample, to study the performance of French and German translations of the PAS, and to determine the power of PAS scales to discriminate dementia and depression diagnosed to DSM-IV criteria. Design and measures The measures were gathered as part of a large community survey. Responses to PAS items were obtained in the course of the administration of the 'Canberra Interview for the Elderly'. Demographic variables, the mini-mental state examination and measures of premorbid intelligence robust to current intellectual impairment were also gathered in the course of the survey. SETTING: A community survey in the cities of Zurich and Geneva, Switzerland. PARTICIPANTS: Subjects were residents of the two cities aged 65 years or older. There were 465 participants from Zurich and 456 from Geneva. Interviews with an informant who knew the subject well were also obtained. Subjects from Zurich were interviewed in German, subjects from Geneva in French. RESULTS: Confirmatory factor analyses of PAS scales showed close agreement between sites and with results obtained in the sample used to construct the instrument. Correlations with demographic and validating variables also showed this pattern. The PAS cognitive impairment and cognitive decline scales were shown to be good discriminators of DSM-IV dementia while the depression scale discriminated DSM-IV major depressive episode. CONCLUSIONS: This study supports the PAS as a valid and reliable summary of the status of older persons with respect to the impairments, changes and symptomatology that lie at the core of dementing and depressive disorders. This study not only supports the results found in the original development sample, but demonstrates that it performs well against DSM-IV diagnostic criteria and amongst speakers of French and German.

Aged↗
PubMed2005

[Endodontic treatment of primary teeth. Pulp exposure and pulp necrosis].

With management of the deep caries in primary teeth we have to take account into the coping strategies of the patient and the state of the development of the dentition. That's why in most cases a root canal treatment of primary incisors or even a pulpotomy is not indicated. Often Intellectual Decision Not To Restore is a good alternative for treatment of deep caries in primary incisors. In deep caries lesions of primary canines and molars preferably minimal invasive techniques as indirect pulp capping are performed. In case of a exposure, the dentist can choose between several types of treatment. Improved techniques have lead to clinical satisfying results of the calcium hydroxide pulpotomy. A partial pulpotomy is if possible the treatment of choice. A resin modified glass ionomer cement is used to cover the pulp wound because it has good sealing properties and it is easy to handle. To limit the burden in young children a root canal treatment in primary teeth is seldom indicated. Overfilling with calcium hydroxide in root canal treatment of primary teeth never causes problems.

Child↗
PubMed1995

Posatirelin for the treatment of late-onset Alzheimer's disease: a double-blind multicentre study vs citicoline and ascorbic acid.

INTRODUCTION: Posatirelin (L-pyro-2-aminoadipyl-L-leucil-L-prolinamide) a new synthetic tripeptide with cholinergic, catecholaminergic and neurotrophic properties, was investigated in the treatment of Alzheimer's disease. MATERIAL AND METHODS: A multicentre, double-blind study vs citicoline (reference drug) and ascorbic acid (inactive drug) was carried out in elderly patients suffering from late-onset Alzheimer's disease. The once daily intramuscular treatment lasted for three months followed by one-month oral treatment with a placebo. Subscales and factors of GBS (Gottfries-Bråne-Steen) Rating Scale were identified as primary measures for efficacy assessment. RESULTS: At the end of the treatment, GBS subscale and factor scores assessing intellectual and emotional impairments, orientation and memory, ability to perform activities of daily living, depression-anxiety, attention and motivation were improved in the postatirelin group, showing significant differences with respect to the citicoline and/or ascorbic acid groups. Tolerability was good in all groups. CONCLUSIONS: The improvement in the GBS Rating Scale score observed in the posatirelin group is clinically relevant. It is worth verifying the drug-induced functional improvements, in further studies with large samples.

Aged↗
PubMed1999

LETM1, a novel gene encoding a putative EF-hand Ca(2+)-binding protein, flanks the Wolf-Hirschhorn syndrome (WHS) critical region and is deleted in most WHS patients.

Deletions within human chromosome 4p16.3 cause Wolf-Hirschhorn syndrome (WHS), which is characterized by severe mental and developmental defects. It is thought that haploinsufficiency of more than one gene contributes to the complex phenotype. We have cloned and characterized a novel gene (LETM1) that is deleted in nearly all WHS patients. LETM1 encodes a putative member of the EF-hand family of Ca(2+)-binding proteins. The protein contains two EF-hands, a transmembrane domain, a leucine zipper, and several coiled-coil domains. On the basis of its possible Ca(2+)-binding property and involvement in Ca(2+) signaling and/or homeostasis, we propose that haploinsufficiency of LETM1 may contribute to the neuromuscular features of WHS patients.

Amino Acid Sequence↗
PubMedDate not supplied

Reliability of the Matson Evaluation of Drug Side Effects Scale (MEDS).

The potential negative effects of psychoactive medication are well documented. Given the high rate of their use in persons with mental retardation, the need to assess and identify these negative effects is great. The Matson Evaluation of Drug Side Effects (MEDS) was designed to evaluate commonly identified side effects with a psychometrically sound checklist. The initial psychometric properties of this scale are presented and discussed. An examination of interrater reliability and internal consistency revealed that the MEDS has excellent consistency across raters and good internal consistency. Potential uses for the scale and directions for future research are reviewed as well.

Adult↗
PubMed1978

Small metacentric nonsatellited extra chromosome: report of five mentally retarded individuals and review of literature. Contribution to further delineation of a new syndrome.

Five mentally retarded male patients with a supernumerary small metacentric nonsatellited chromosome were found to have many clinical features in common. The face showed characteristic small crowded features, the bodily habitus was asthenic, and the hands and feet had minor abnormalities. Renal anomalies were present in two patients. One patient had a myelomeningocele. Cytogenetic studies employing Q, R, and C banding in four patients showed the small extra chromosome to have staining properties compatible with an isochromosome of the short arm of chromosome 18. A comparison with previous case reports suggests a new syndrome. However, the identity of the extra chromosome has not yet been determined.

Adolescent↗
PubMed1975

Isolation and identification of 2-deoxy-2,3-dehydro-N-acetylneuraminic acid from the urine of a patient with sialuria.

N-Acetylneuraminic acid preparations from the urine of a patient with sialuria contain 1--2% of 2-deoxy-2,3-dehydro-N-acetylneuraminic acid. This new human sialic acid was isolated by ion-exchange and partition chromatography. The structure has been elucidated by mass spectrometry and confirmed by comparison with the synthetic compound. The properties of this unsaturated sialic acid in the orcinol/Fe3+/HCl and the periodic acid/thiobarbituric acid tests as well as in thin-layer and gas-liquid chromatography are described. It does not react with acylneuraminate pyruvatelyase. The origin of this new human sialic acid is discussed.

Carbohydrate Metabolism, Inborn Errors↗
PubMed1983

[Problem of defining psychopathies and so-called preclinical states in borderline psychiatry].

The structure of the personality of mentally deficient law violators of sound mind can be differentiated into two types of aberrations, namely pathological (clinical disturbances) and nonpathological (social and psychologic deformations). These properties in different combinations were detected in all law violators examined. The role of these deviations in the genesis of law violation is discussed. Differential assessment of clinical, social and psychological phenomena allows a more clear-cut differentiation of psychopathic, psychopathlike and some preclinical conditions and also determination of their probable time-course.

Antisocial Personality Disorder↗
PubMed2001

Methodological issues in interviewing and using self-report questionnaires with people with mental retardation.

In this article the authors review methodological issues that arise when interviews and self-report questionnaires are used with people with mental retardation and offer suggestions for overcoming some of the difficulties described. Examples are drawn from studies that use qualitative methodology, quantitative studies assessing different question types, and studies reporting on the development of instruments measuring psychiatric symptoms, self-concept, and quality of life. Specific problems that arise with respect to item content (e.g., quantitative judgments, generalizations), question phrasing (e.g., modifiers), response format (e.g., acquiescence, multiple-choice questions), and psychometric properties (factor structure and validity) are discussed. It is argued that because many self-report questionnaires include questions that have been found to be problematic in this population, more attention needs to be paid to establishing the validity of such measures and to clearly defining the population for which the instrument is designed.

Adult↗
PubMed2004

The fragile X mental retardation protein has nucleic acid chaperone properties.

The fragile X syndrome is the most common cause of inherited mental retardation resulting from the absence of the fragile X mental retardation protein (FMRP). FMRP contains two K-homology (KH) domains and one RGG box that are landmarks characteristic of RNA-binding proteins. In agreement with this, FMRP associates with messenger ribonucleoparticles (mRNPs) within actively translating ribosomes, and is thought to regulate translation of target mRNAs, including its own transcript. To investigate whether FMRP might chaperone nucleic acid folding and hybridization, we analysed the annealing and strand exchange activities of DNA oligonucleotides and the enhancement of ribozyme-directed RNA substrate cleavage by FMRP and deleted variants relative to canonical nucleic acid chaperones, such as the cellular YB-1/p50 protein and the retroviral nucleocapsid protein HIV-1 NCp7. FMRP was found to possess all the properties of a potent nucleic acid chaperone, requiring the KH motifs and RGG box for optimal activity. These findings suggest that FMRP may regulate translation by acting on RNA-RNA interactions and thus on the structural status of mRNAs.

Amino Acid Motifs↗
PubMedDate not supplied

Iron and copper metabolism.

Iron and copper are essential nutrients, excesses or deficiencies of which cause impaired cellular functions and eventually cell death. The metabolic fates of copper and iron are intimately related. Systemic copper deficiency generates cellular iron deficiency, which in humans results in diminished work capacity, reduced intellectual capacity, diminished growth, alterations in bone mineralization, and diminished immune response. Copper is required for the function of over 30 proteins, including superoxide dismutase, ceruloplasmin, lysyl oxidase, cytochrome c oxidase, tyrosinase and dopamine-beta-hydroxylase. Iron is similarly required in numerous essential proteins, such as the heme-containing proteins, electron transport chain and microsomal electron transport proteins, and iron-sulfur proteins and enzymes such as ribonucleotide reductase, prolyl hydroxylase phenylalanine hydroxylase, tyrosine hydroxylase and aconitase. The essentiality of iron and copper resides in their capacity to participate in one-electron exchange reactions. However, the same property that makes them essential also generates free radicals that can be seriously deleterious to cells. Thus, these seemingly paradoxical properties of iron and copper demand a concerted regulation of cellular copper and iron levels. Here we review the most salient characteristics of their homeostasis.

Copper↗
PubMed2003

A pathology of the animal spirits -- the clinical neurology of Thomas Willis (1621-1675). Part II -- disorders of intrinsically abnormal animal spirits.

Thomas Willis (1621-1675), author of the classical work Cerebri Anatome (1664), was arguably the father of the modern era of neurology. His clinical neurology, as described in his Pathologiae Cerebri (1667) and De Anima Brutorum (1672), was largely derived from personal observations and not from traditional authorities and was based around his concept of the animal spirits, a fictitious entity in many ways analogous to the present day idea of the nerve impulse. This concept allowed him to develop a pathology of the animal spirits which embraced the whole content of the clinical neurology and psychiatry of his times. The anatomical and physiological background to Willis' concepts of animal spirit dysfunction, and those disorders he regarded as due to disturbed function of intrinsically normal animal spirits, have been dealt with in the previous part of this paper. The disorders he attributed to intrinsically abnormal animal spirits, dealt with in this part of the paper, comprised two categories. In one, the animal spirits possessed explosive properties, whilst in the other the abnormalities were non-explosive in their nature. The former category included epilepsy, hysteria and hypochondriasis, whilst the latter included mainly disorders now considered psychiatric e.g. delirium, melancholy, madness and stupidity. Willis' ideas about the pathogenesis of nervous system disorder seem never to have been generally accepted, partly because they appeared at a time when others were increasingly calling into question the existence of the animal spirits. Nevertheless, Willis' attempt to record and interpret all nervous system disease on the basis of disorder of function of a single underlying mechanism represents a formidable synthetic intellectual endeavour on the part of a very busy physician.

Animals↗
PubMed1992

Mental impairment in the West Midlands.

The case records of 55 people from the West Midlands (UK) fulfilling the Mental Health Act 1983 criteria for mental impairment or severe mental impairment, were studied. Most were young men with mild mental retardation. 73 per cent were resident in (or on leave from) mental handicap hospitals, and 27 per cent resident in special hospitals. 29 per cent were subject to a Restriction Order. Most had lived in hospital for more than six years. The commonest problem behaviours were aggression, property offences and inappropriate or offending sexual behaviour. 31 per cent were mentally ill or had a past history of mental illness. A diverse range of services appears necessary to meet the needs of this group of people.

Adolescent↗
PubMed1988

The mentally handicapped criminal offender. A 10-year study of two hospitals.

With mentally handicapped people now being moved into the community, information on mentally handicapped criminal offenders is valuable. A group of such offenders was examined by reviewing the case-notes of 92 patients referred on hospital order to two mental-handicap hospitals over 10 years. Compared with the general criminal population, these offenders' ages were higher, the ratio of male to female offenders was similar, and the proportion of married people was lower. The offences committed were for the most part serious, with a greater number of offences against property and public order in the subgroup whose intelligence were in the mentally handicapped range. Their tested intelligence fell almost entirely into the normal and mild mental-handicap range, suggesting that factors other than intelligence testing, such as social skills, were considered in their admission to the hospitals.

Adult↗