A four year prospective study of age-related cognitive change in adults with Down's syndrome

BACKGROUND: While neuropathological studies indicate a high risk for Alzheimer's disease in adults with Down's syndrome, neuropsychological studies suggest a lower prevalence of dementia. In this study, cognitive deterioration in adults with Down's syndrome was examined prospectively over 4 years to establish rates and profiles of cognitive deterioration. METHODS: Fifty-seven people with Down's syndrome aged 30 years or older were assessed using a battery of neuropsychological tests on five occasions across 50 months. Assessments of domains of cognitive function known to change with the onset of Alzheimer related dementia were employed. These included tests of learning, memory, orientation, agnosia, apraxia and aphasia. The individual growth trajectory methodology was used to analyse change over time. RESULTS: Severe cognitive deterioration, such as acquired, apraxia and agnosia, was evident in 28.3% of those aged over 30 and a higher prevalence of these impairments was associated with older age. The rate of cognitive deterioration also increased with age and degree of pre-existing cognitive impairment. Additionally, deterioration in memory, learning and orientation preceded the acquisition of aphasia, agnosia and apraxia. CONCLUSIONS: The prevalence of cognitive impairments consistent with the presence of Alzheimer's disease is lower than that suggested by neuropathological studies. The pattern of the acquisition of cognitive impairments in adults with Down's syndrome is similar to that seen in individuals with Alzheimer's disease who do not have Down's syndrome.     

Glucose effects on cognition in adults with Down's syndrome.

Glucose enhances memory in a variety of individuals, including people with Alzheimer's disease. By 35 years of age, adults with Down's syndrome (DS) develop the characteristic plaques and tangles found in Alzheimer's disease, despite findings indicating that not all older DS individuals meet criteria for dementia. To examine the possibility that glucose enhances memory in adults with DS (mean age?=?35 years, range?=?19–55 years), adults with DS were given a battery of tests specifically designed for individuals with DS in glucose and control conditions. No participant met criteria for dementia, regardless of age. Glucose enhanced performance on tests requiring both long-term memory and auditory processing. In addition, increased age was associated with poorer performance on the majority of tests in the control condition, indicating that cognitive decline with aging may be more prevalent in DS than previously believed. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Hemispheric asymmetry in Down's Syndrome children.

Examined auditory processing of linguistic stimulation presented dichotically to groups of 30 Down's Syndrome (DS) children, 20 non-DS retarded children who were MA and CA matched, and 20 MA matched nonretarded children, all of whom were right-handed. Ss completed a knowledge-of-numbers test and the Slosson Intelligence Test. They then listened to 20 trials of a dichotic message at approximately 70–75 db and repeated digits they heard. DS Ss showed a significant left ear advantage in processing the auditory linguistic material, while the other Ss showed the normal right ear advantage. Findings corroborate that having DS has a specific effect on the functional organization of the CNS auditory system. The right hemisphere may not be as efficient as the left hemisphere at language processing. (29 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Hyperthyroidism in a girl with Down's syndrome

We report an adolescent girl with Down's syndrome, who presented with hyperthyroidism. Autoimmune thyroid disorders can occur in children with Down's syndrome, hypothyroidism developing more frequently. Hyperthyroidism can also be associated with Down's syndrome, and should not be missed.     

Interplay among behavioral systems: Illustrations from the study of attachment, affiliation, and wariness in young children with Down's syndrome.

42 Down's syndrome (DS) children, age 30–42 mo, were seen in M. D. Ainsworth and B. A. Wittig's (1969) "strange situation" to assess the interrelationships among the affiliative, attachment, and fear/wariness behavioral systems. A stranger evoked affiliative behaviors in Ss but also elicited wariness and attachment behaviors. The emergence, sequence, and intensity of these behavioral systems varied with the context and the behaviors of both stranger and mother. The finding that more than one behavioral system was activated suggests that an explanation of social responsiveness must be sufficiently broad and integrative to encompass different but interrelated behavioral systems, with their respective functions and determinants. Despite certain quantitative and qualitative differences, behavioral systems appear to be similarly organized in DS and normal children. It is argued that the behavioral organization manifested by the retarded children supports and extends E. Zigler's (see record 1969-08608-001) "developmental" position. These findings suggest that the DS group constitutes a legitimate target of research for elucidating the processes of normal and deviant development. (43 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Folliculitis in Down's syndrome

Twenty-two male and 20 female adults with Down's syndrome were examined. Ten of the men and two of the women had a follicular rash consistent with Malassezia folliculitis. Oral itraconazole treatment produced a significant improvement in the rash, accompanied by a decrease in the skin Malassezia count. Clinical relapse occurred when therapy was discontinued, and was accompanied by return of the Malassezia yeasts.     

A pilot study of the neuropsychological benefits of computerized cognitive rehabilitation in Ugandan children with HIV.

Objective: Because antiretroviral treatment (ART) fails to improve neurocognitive impairment in children with HIV, we completed a pilot study evaluating the feasibility and cognitive benefit of computerized cognitive rehabilitation therapy (CCRT) in Ugandan children with HIV. Method: Sixty Ugandan children with HIV (23 on ART) were randomly assigned to 10 sessions of Captain's Log CCRT (Sandford, 2007) training configured for attention and memory skills or no intervention. Kaufman Assessment Battery for Children (2nd ed., KABC–2; Kaufman & Kaufman, 2004) performance at baseline indicated pervasive neurocognitive impairment. Cognitive ability was assessed before and after training using the Cogstate computerized neuropsychological test (Darby, Maruff, Collie, & McStephen, 2002). Viral load along with CD4 and CD8 absolute and activation levels also were measured posttest. Results: CCRT was well received with a 95% adherence rate to scheduled training sessions. CCRT intervention children showed greater improvement on a Cogstate card detection task of simple attention (p = .02), and speed of correct moves on a Groton Maze Learning Task (p     

Olfactory function in young adolescents with Down's syndrome

Decreased ability to smell is present in adults with Down's syndrome, many of whom are known to have brain pathology analogous to that seen in Alzheimer's disease. Because olfactory loss is well documented in Alzheimer's disease, the question arises whether young adolescents with Down's syndrome, who have no clear Alzheimer's disease-like neuropathology, also exhibit olfactory dysfunction. To consider this issue, standardised tests of odour discrimination and identification were administered to 20 young adolescents with Down's syndrome (mean age (SD) 13.89 (1.98) years) and their test scores were compared with 20 mentally retarded and 20 non-mentally retarded control subjects matched to the patients with Down's syndrome on the basis of cognitive ability. No significant differences in olfactory function were found among the three study groups. These findings, along with those from studies of olfactory function in older patients with Down's syndrome, suggest that Down's syndrome related olfactory dysfunction occurs only at ages when Alzheimer's disease-like pathology is present.     

Testing parental instructions to schizophrenic and normal children: A pilot study.

Hypothesized that parents communicate to their schizophrenic children in conflicting ways. An experiment was designed where parents instructed their children in a task. Instructions were given from a separate room and were tape recorded so they could be played to matched samples of children. Parents of schizophrenics, of nonschizophrenic abnormal children, and of normal children were contrasted. The achievement of the children in the task was measured, and matched samples of children listened to the instructions of a group of parents of schizophrenics and a group of parents of normal children. Indications were that parents of schizophrenics do not communicate in more conflicting ways than parents of normal children when the measurement is the success of a child in following their instructions. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Home observations of mothers and sibling dyads comprised of Down's syndrome and nonhandicapped children.

Observed interaction in 31 mother–child–child triads in which 1 of the children had Down""s syndrome. Down""s syndrome siblings were all aged 18–117 mo, nonhandicapped siblings were aged 12–132 mo, and mothers were aged 30–46 yrs. Mothers were more positive and directive toward their children with Down""s syndrome but received more positive initiations from their nonhandicapped children. Significant reciprocity between mother and child was found in dyads with a handicapped child but not in dyads with a nonhandicapped child. The handicapped child""s negative behavior toward the sibling was the most important predictor of interaction across the triad. Individual differences in the social behavior of children with Down""s syndrome may have an impact throughout family systems. (French abstract) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

What do babies listen to most? A developmental study of auditory preferences in nonhandicapped infants and infants with Down's syndrome.

Studied the auditory preferences of 10 Down's syndrome (DS; mean CA 12.4 mo; mean MA 9.3 mo) and 10 nonhandicapped (NH; mean CA 9.3 mo; mean MA 9.6 mo) infants at the developmental ages of around 9 and 18 mo. An apparatus that allows infants to choose to listen to 1 of a pair of auditory stimuli and that records their responses was used. At both ages, Ss had the choice of listening first to either a children's rhyme or a repetitive tone and then to either the mother talking to her own child (baby talk) or the mother talking to an adult. At both ages, both groups of Ss significantly preferred to listen to the children's rhyme and to the baby talk. At 9 mo, both groups listened significantly more to nursery rhymes than to spoken stimuli; by 18 mo, NH Ss showed a significant increase in listening to baby talk, which they then listened to longer than to rhymes. DS Ss showed a significant decrease in listening to baby talk at this age and still listened longer to rhymes. (12 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Motivational interviewing with cocaine-dependent patients: A pilot study.

A brief motivational interviewing (MI) intervention was evaluated within the context of an outpatient, cocaine-detoxification program. MI was hypothesized to assist patients in completing the detoxification program and to improve outcomes during subsequent treatment. Participants (N = 105) were randomly assigned to MI or to detox-only conditions. Results indicated that although participants completed the detoxification program at equal rates, completers who received MI increased use of behavioral coping strategies and had fewer cocaine-positive urine samples on beginning the primary treatment. MI patients with lower initial motivation were more likely to complete detoxification. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Histomorphometric study in children with idiopathic nephrotic syndrome

There is agreement on the clinical diagnostic criteria for acute inflammatory demyelinating polyneuropathy (AIDP/GBS) however, there is lack of consensus for detection of demyelination. In order to critically evaluate the prevailing criteria, sixty-six patients who fulfilled NINCDS criteria and had typical features of GBS were studied for electrophysiological abnormalities of peripheral nerves by using standard methods (median, common peroneal, sural and ulnar) between 1 to 12 weeks after the onset of symptoms. The commonest abnormality on motor nerve conduction study was prolonged distal latency (75%-83%) followed by reduction in CMAP amplitude (63%-82%), decreased velocity (48%-62%), conduction block (17%-39%) and f-wave abnormalities (37.8%-59%). Sensory conduction abnormalities were detected in over 20% of median, 25% of ulnar and 33% of sural nerves. All the patients had abnormality of at least two motor conduction parameters in one nerve when values beyond 2 SD of the mean were considered abnormal and over 70% of patients had three abnormalities in two nerves or two abnormalities in three nerves. Comparison with the prevailing criteria for demyelination revealed that the number of patients fulfilling them varied widely: Albers et al. (1985): 74.2%, Albers et al. (1989): 40.9% and Cornblath: 30.3%. We believe that the current criteria for detection of demyelination in acute neuropathy are too strict, underestimate the underlying pathology in GBS and need reassessment.     

Dichotic ear advantages in adults with Down's syndrome predict speech production errors.

Intellectually challenged adults, with and without Down's syndrome (DS), pointed to drawings of animals following the dichotic presentation of animal names. Although there were no reliable ear differences between the 2 groups, there was tremendous between-persons variability within the group of participants with DS. Moreover, left-ear advantages in persons with DS were associated with more speech production errors when participants either read or repeated a string of 1 syllable words. This was not true for control participants. The relationship between ear advantage and speech errors in persons with DS could reflect their unique pattern of cerebral specialization and brain development. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

A neurophysiological study in children and adolescents with Crigler-Najjar syndrome type I

BACKGROUND: Paclitaxel, a natural product from Taxus brevifolia, is a microtubule stabilizing agent, which has been shown to block different cells in the G2/M phase of the cell cycle and consequently, to modulate their radioresponsiveness. Our aim was to test the cytotoxic and radiosensitizing potential of paclitaxel, with respect to different gynecological tumors with varying radiosensitivities. MATERIAL AND METHOD: We performed clonogenic assays and flow cytometry on 2 cell lines, MCF-7 (breast) and CaSki (cervix) cells, and on 2 primary ovarian tumor samples (OC-I and OC-II). The cells were irradiated with 200 kV X-rays, radiation doses of up to 8 Gy were applied either as single doses or in 2 Gy fractions. Paclitaxel concentrations varied from 0.07 to 700 nM, incubation times varied from 3 to 120 h. RESULTS: Paclitaxel alone changed the cell cycle distribution of the cells tested and was cytotoxic in a time and concentration dependent manner. When combined with radiation, most schedules resulted in additive effects of the combined treatments. However, for MCF-7 cells, when 7 nM paclitaxel, applied 24 h before irradiation, were combined with fractionated irradiation a supra-additive effect with a SER of 1.2 was found. For CaSki cells, under comparable conditions the SER was 1.13 but the effects were not statistically significant. CONCLUSION: Under specific conditions, paclitaxel exerted a weak radiosensitizing effect on breast and cervical carcinoma cells. A therapeutic gain may be possible on the basis of an optimal paclitaxel/radiation scheduling.