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Results for “Intellectual Disability”

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PubMed1996

Specificity and developmental consequences of speech loss in children with pervasive developmental disorders.

Speech loss (SL) was compared in 276 children who had pervasive developmental disorders (PDD) with 62 children with intellectual disabilities without PDD. Speech loss seems relatively specific to PDD because it is significantly more common in children with PDD (26.1%, 72/276) than in those with intellectual disabilities (1.6%, 1/62). In three PDD categories, speech loss occurred in all the 12 children with disintegrative psychosis, 35/149 (23.5%) children with infantile autism and 25/115 (21.7%) children with other PDD. Children with pervasive developmental disorders and speech loss had spoken significantly earlier yet developed less satisfactorily after speech loss than those without it. Speech loss seems fairly specific to PDD and is indicative of unfavorable intellectual development in children with PDD.

Adolescent↗

PubMed2003

Assessment of change with the Developmental Behaviour Checklist.

BACKGROUND: The Developmental Behaviour Checklist (DBC) is a 96-item instrument designed for assessing behavioural and emotional problems among young people with intellectual disability. METHODS: The present study investigated the validity of the DBC as a measure of change. Changes in individual's DBC scores were correlated with changes in expert clinicians' ratings of the same subjects. RESULTS: A high correlation was found between expert clinician ratings of change in behaviour and change in the total behaviour problem score of the DBC. CONCLUSIONS: The DBC is able to measure changes in behaviour and emotions which are recognized by an experienced clinician who is following the progress of a child with ID.

Adolescent↗

PubMed2004

Involving users with learning difficulties in health improvement: lessons from inclusive learning disability research.

In this paper the author considers the lessons to be drawn from what is termed "inclusive" learning disability research for user involvement around health improvement. Inclusive learning disability research refers to research where people with learning difficulties (intellectual disability) are involved as active participants, as opposed to passive subjects. There is by now a considerable body of such research, developed over the past 25 years. From the review, the author draws attention to areas which can inform practice in involvement of users in a way that adds value.

Decision Making, Organizational↗

PubMedDate not supplied

Alcohol and criminal behaviour.

Substance abuse is a factor in some but not all incidents of crime: more than half of murders are committed when the killer is intoxicated. Males are more likely than females to be violent when consuming alcohol, and a past history of violence is predictive of future crime. The risk of violent behaviour is greater in subjects with dual diagnosis (e.g. alcohol dependence and psychiatric disorders). Offenders with an intellectual disability are overrepresented in prison and court populations, and more than 50% of them have a problem with alcohol. Since these subjects, in addition to intellectual disability and alcohol use, have psychiatric and behavioural problems, early recognition, in an effort to prevent future crimes, is an appropriate goal. Specific programmes have to address their particular needs.

Alcohol Drinking↗

PubMed2002

Screening young people for autism with the developmental behavior checklist.

OBJECTIVE: To determine whether a subset of items from the Developmental Behavior Checklist (DBC) could be selected to construct a reliable autism screening tool. METHOD: A 29-item scale-the Developmental Behavior Checklist-Autism Screening Algorithm (DBC-ASA)-was developed by using items from the DBC and evaluated in a sample comprising 180 children who met criteria for autism and 180 controls matched for age, sex, and IQ range. RESULTS: This study found that the DBC-ASA has good validity in discriminating young people (4-18 years) with autism and IQ ranging from normal to severe intellectual disability from others using a cutoff score of 17. CONCLUSION: The DBC-ASA is an effective autism screening questionnaire for at-risk young people, including those with intellectual disability.

Adolescent↗

PubMed1997

Early menopause in women with Down's syndrome.

We used the AAMR's Adaptive Behavior Scale to ascertain current menstrual status in a population-based sample of 157 women with Down's syndrome (DS) and 187 women with other intellectual disability, all 40 years of age or older. The age-adjusted likelihood of menopause was twice as high in women with DS syndrome as in women with other intellectual disability (OR = 2.3; 95% CI = 1.1-4.9). Treated thyroid conditions did not influence menstrual status and did not modify the relationship between DS and menstrual status. These findings support the hypothesis that women with DS experience menopause at an earlier age and that this may be associated with accelerated aging.

Adult↗

PubMed2006

Clinical and molecular cytogenetic characterisation of a newly recognised microdeletion syndrome involving 2p15-16.1.

BACKGROUND: During whole genome microarray-based comparative genomic hybridisation (array CGH) screening of subjects with idiopathic intellectual disability, we identified two unrelated individuals with a similar de novo interstitial microdeletion at 2p15-2p16.1. Both individuals share a similar clinical phenotype including moderate to severe intellectual disability, autism/autistic features, microcephaly, structural brain anomalies including cortical dysplasia/pachygyria, renal anomalies (multicystic kidney, hydronephrosis), digital camptodactyly, visual impairment, strabismus, neuromotor deficits, communication and attention impairments, and a distinctive pattern of craniofacial features. Dysmorphic craniofacial features include progressive microcephaly, flat occiput, widened inner canthal distance, small palpebral fissures, ptosis, long and straight eyelashes, broad and high nasal root extending to a widened, prominent nasal tip with elongated, smooth philtrum, rounding of the upper vermillion border and everted lower lips. METHODS: Clinical assessments, and cytogenetic, array CGH and fluorescence in situ hybridisation (FISH) analyses were performed. RESULTS: The microdeletions discovered in each individual measured 4.5 Mb and 5.7 Mb, spanning the chromosome 2p region from 57.2 to 61.7 Mb and from 56 to 61.7 Mb, respectively. Each deleted clone in this range demonstrated a dosage reduction from two to one copy in each proband except for clone RP11-79K21, which was present in three copies in each proband and in four copies in their respective parents (two per each chromosome 2 homologue). DISCUSSION: The common constellation of features found in the two affected subjects indicates that they have a newly recognised microdeletion syndrome involving haploinsufficiency of one or more genes deleted within at least a 4.5-Mb segment of the 2p15-16.1 region.

Abnormalities, Multiple↗

PubMed2006

California community residential facilities for individuals with intellectual and developmental disabilities.

Evolving residential requirements for individuals with mild and moderate intellectual disabilities and related developmental disabilities increasingly place these people in community settings. The increasing numbers of these individuals are dependent upon local practitioners for needed health services. National and California data are reviewed in an effort to provide a general awareness of these community living arrangements, which in turn may assist in the delivery and the follow-up of oral health services.

California↗

PubMed1998

Social class differences in health until the age of seven years among the Finnish 1987 birth cohort.

Studies on social class differences in childhood health are controversial partly because of different data collection methods, limited sample sizes and the use of limited numbers of health indicators. The increasing collection of health register data enables the use of such data in social class studies. Our purpose was to investigate social class differences in mortality and morbidity among all children born in Finland in 1987 (N=59,865 liveborns) until the age of seven by using several national health registers, and to study whether perinatal health explains these differences. The follow-up was based on data linkage with six national health registers, with 18 regional registers of mentally disabled children, covering the whole country, and with 38 educational registers of the largest county. Morbidity was measured in terms of a cumulative disease index, the cumulative incidence of asthma, diabetes, epilepsy and intellectual disability, hospitalisations, disease-related welfare benefits and special education. Social class, divided in four groups (I-III, Others) was defined by using the mother's occupation at the time the child was seven years old. Our study showed that register-based data collection is a feasible method for studying social class differences in health. In the unadjusted analysis, social class differences were found for all indicators except mortality after the age of one year and for the cumulative incidence of asthma and diabetes. After adjusting for confounders, the children in the lowest social class had the highest risk for poor health outcome both in the perinatal period and in childhood, and had the most intellectual disabilities, the highest mean of hospitalisation days, and received the most special education. The differences were not explained by perinatal health. The health of the children in the lowest social class was poorer, especially regarding mental indicators.

Chi-Square Distribution↗

PubMed2005

Brief report: parental descriptions of sleep problems in children with autism, Down syndrome, and Prader-Willi syndrome.

Children with an intellectual disability (ID) are at high risk of developing sleep problems. The extent to which the prevalence and nature of sleep problems in these children is dependent on the disorder underlying their intellectual impairments remains unclear. This study examined and compared parental descriptions of sleep problems in children with autism (n = 37), Down syndrome (DS; n = 15), Prader-Willi syndrome (PWS; n = 29), presumed familial intellectual disability (FID; n = 29), and typically developing children (TD; n = 55) in order to determine any influences of disorder on sleep patterns. The prevalence of sleep problems in the disability groups was at least four times higher than for TD children. Sleep problems were more prevalent in autism than the other disorders. Settling difficulties and co-sleeping were more common in the children with autism, whereas sleep maintenance problems were common in autism, DS, and FID, and daytime napping and excessive daytime sleepiness differentiated the children with PWS. These findings are discussed in light of the specific disorders, and with respect to the impact that sleep problems can have on the child and his/her family.

Adolescent↗

PubMed2006

Severe myoclonic epilepsy of infancy (Dravet syndrome): recognition and diagnosis in adults.

Establishing an etiologic diagnosis in adults with refractory epilepsy and intellectual disability is challenging. We analyzed the phenotype of 14 adults with severe myoclonic epilepsy of infancy. This phenotype comprised heterogeneous seizure types with nocturnal generalized tonic-clonic seizures predominating, mild to severe intellectual disability, and variable motor abnormalities. The diagnosis was suggested by a characteristic evolution of clinical findings in the first years of life. Ten had mutations in SCN1A and one in GABRG2.

Adolescent↗

PubMed1992

Communication abilities and Rett syndrome.

Investigated 6 girls with Rett syndrome (RS), ages between 2 and 13 years, to provide comprehensive descriptions of their communicative behaviors. Previous studies have not focused on communicative competence nor on the intentionality of the language used by children with RS. This study concluded that all members of the group investigated were at a preintentional level of communication. Intentional communication has previously been reported to develop in normally developing and intellectually disabled children attaining Piagetian Sensorimotor Stage V in Means-End behavior. The present study also investigated the cognitive performance of the group. We concluded that the preintentional level of communication noted was consistent with the subjects' profound intellectual disability. No Means-End (i.e., purposeful) behavior beyond Piagetian Sensorimotor Stage III could be elicited from the 6 girls. The relevance of this study for therapeutic intervention is discussed.

Adolescent↗

PubMed2006

Is theory of mind understanding impaired in males with fragile X syndrome?

Males with fragile X syndrome (FXS) have difficulties with social interaction and many show autistic features. This study examined whether the social deficits characteristic of FXS are associated with theory of mind difficulties. Two groups of boys with FXS participated: a group with few autistic features and a group with many autistic features. An intellectual disability control group also participated. In addition to using standard theory of mind tasks, new techniques were used that were able to separate out the various processing demands of the task (e.g., memory, inhibitory control). Overall, the findings indicate that both groups of boys with FXS have difficulty with theory of mind tasks compared to an intellectual disability control group. However, both groups with FXS also performed worse on comparison trials that required working memory but not theory of mind. Theory of mind difficulties are likely to be an important aspect of the FXS clinical profile, but are most likely the result from a more basic difficulty with working memory.

Adolescent↗

PubMed2005

Clinical manifestations of a unique X-linked retinal disorder in a large New Zealand family with a novel mutation in CACNA1F, the gene responsible for CSNB2.

PURPOSE: To describe the phenotype in a New Zealand family with an unusual severe X-linked retinal disorder with a novel I745T mutation in CACNA1F, the gene responsible for incomplete congenital stationary night blindness (CSNB2). METHODS: Members of the family tree were invited for clinical, psychophysical and electrodiagnostic evaluation. RESULTS: Male family members had severe non-progressive visual impairment, abnormal colour vision, congenital nystagmus, hyperopia and normal fundi. Some were intellectually disabled. Female family members had congenital nystagmus and decreased visual acuity frequently associated with high myopia. Electroretinograms (ERG) identified reduced rod and cone responses with negative waveform in male and female family members, with atypical features for CSNB2. CONCLUSIONS: Although there were similarities to CSNB2, distinctive features in male family members included severity of phenotype, and association of intellectual disability. Moreover, all female heterozygotes had clinical and ERG abnormalities. CACNA1F encodes the Ca(v)1.4 alpha1 subunit of a voltage-gated calcium channel, which may mediate neurotransmitter release from photoreceptors. Molecular analyses, reported separately, identified a novel I745T CACNA1F mutation that was associated in vitro with major alterations in gating and kinetics of the Ca(v)1.4 channel. It is speculated that the unique phenotype described in this family may reflect similarly altered function of Ca(v)1.4 channel activity in vivo.

Adolescent↗

PubMed1997

Self-injurious behaviour and body site preference.

Self-injury by people with intellectual disabilities is a highly problematic and damaging behaviour with profound implications for quality of life. To date, very little detailed descriptive information has been available on the distribution and location of body sites that are injured. This study presents preliminary information on the locations of the self-injury body sites of 29 school-age individuals with developmental and intellectual disabilities who self-injured daily. Teaching staff in school-based special education programmes independently recorded the body locations of where their student's self-injured. Approximately 80% of the reported self-injury was directed disproportionately toward the head and hands. Three-quarters of head-directed self-injury was located on the front of the head, and 83% of hand-directed self-injury was located on the back of the hands. Furthermore, 32% of the body sites toward which self-injury was directed were located on stimulation-produced analgesia body sites. The implications of these findings are discussed in relation to the opioid hypothesis of self-injurious behaviour, and with regard to future research investigating functional diagnostic strategies considering both social and biological variables.

Acupuncture Therapy↗

PubMed2004

Compulsive behavior and eye blink in Prader-Willi syndrome: neurochemical implications.

Compulsive behavior in Prader-Willi syndrome is well-documented, though the neurochemical basis of these behaviors remains unknown. We studied a group of 16 people with Prader-Willi syndrome and a comparison group of 19 people with intellectual disability. Using eye-blink rate as an indirect measure of central nervous system dopamine, we found a higher eye-blink rate in the Prader-Willi syndrome group and a relationship between compulsive behavior and eye-blink rate across both groups. Our findings indicate a relationship between compulsions and an indirect measure of dopamine in people with intellectual disability. Our findings may provide evidence for a gene-brain-behavior link between dopamine levels and GABAergic mechanisms in individuals with Prader-Willi syndrome.

Adolescent↗

PubMed2004

Older parent caregivers' engagement with the service system.

Older parents of adults with intellectual disability are reported to be frequently isolated from the services designed to support their caregiving. The interaction between older parent caregivers' biographies and their involvement with the service system was examined. Parental status was predicted to be an explanatory mechanism for understanding the differential nature of their engagement with services. Analysis of interviews with 64 such caregivers of adults with intellectual disability revealed that their perceptions of their parental status were derived from a complex interaction of their values and beliefs, actions in relation to their adult child, constraints as well as resources available to them, and their relationships with service agencies. Implications for service delivery and directions for research are discussed.

Aged↗

PubMed2004

Mother-Baby psychiatric units (MBUs): national data collection in France and in Belgium (1999-2000).

The French version of the Marcé checklist was used to collect data for 176 joint admissions to 11 psychiatric mother-baby units in 1999 and 2000. Mean age of the babies at admission ranged from 4 to 16 weeks. Two units also admitted older children. Mothers admitted were diagnosed with schizophrenia or chronic delusional disorders (n = 44), acute transitory psychosis "Bouffée délirante" (n = 20), bipolar disorders (n = 20), depressive illness (n = 38), personality disorders or intellectual disability (n = 39), and other disorders (n = 15). The mean duration of hospitalisation was 11 weeks. Units that also offered day-care admission in the same or a near-by unit had shorter mean admissions. More than half the women's partners (or babies' fathers) had mental health problems. Women with schizophrenia or chronic delusional disorders and personality disorders or intellectual disability remained hospitalised longer, improved less, and were more often separated from their babies, or discharged with supervision, than women admitted with other diagnoses.

Adult↗