Health needs of women with mental retardation and developmental disabilities.

Comments that the Women's Health Initiative described by K. A. Matthews (see record 1997-02873-001) should be expanded to include research on underserved populations, especially women with mental retardation and developmental disabilities. It is stated that the goal should be to study the health care needs of this population across the life span, not just in the postmenopausal phase of life. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Bilateral periventricular nodular heterotopia with mental retardation and syndactyly in boys: a new X-linked mental retardation syndrome

Bilateral periventricular nodular heterotopia (BPNH) is a recently recognized malformation of neuronal migration, and perhaps proliferation, in which nodular masses of gray matter line the walls of the lateral ventricles. Most affected individuals have epilepsy and normal intelligence with no other congenital anomalies. A striking skew of the sex ratio has been observed because 31 of 38 probands have been female, and one gene associated with BPNH was recently mapped to chromosome Xq28. We report three unrelated boys with a new multiple congenital anomaly-mental retardation syndrome that consists of BPNH, cerebellar hypoplasia, severe mental retardation, epilepsy, and syndactyly. Variable abnormalities included focal or regional cortical dysplasia, cataracts, and hypospadius. We hypothesize that this syndrome involves the same Xq28 locus as isolated BPNH, and we review the expanding number of syndromes associated with BPNH.     

De novo truncation of chromosome 16p and healing with (TTAGGG)n in the alpha-thalassemia/mental retardation syndrome (ATR-16)

We have previously described a series of patients in whom the deletion of 1-2 megabases (Mb) of DNA from the tip of the short arm of chromosome 16 (band 16p13.3) is associated with alpha-thalassemia/mental retardation syndrome (ATR-16). We now show that one of these patients has a de novo truncation of the terminal 2 Mb of chromosome 16p and that telomeric sequence (TTAGGG)n has been added at the site of breakage. This suggests that the chromosomal break, which is paternal in origin and which probably arose at meiosis, has been stabilized in vivo by the direct addition of the telomeric sequence. Sequence comparisons of this breakpoint with that of a previously described chromosomal truncation (alpha alpha)TI do not reveal extensive sequence homology. However, both breakpoints show minimal complementarity (3-4 bp) to the proposed RNA template of human telomerase at the site at which telomere repeats have been added. Unlike previously characterized individuals with ATR-16, the clinical features of this patient appear to be solely due to monosomy for the terminal portion of 16p13.3. The identification of further patients with "pure" monosomy for the tip of chromosome 16p will be important for defining the loci contributing to the phenotype of this syndrome.     

Are individuals with severe mental retardation and multiple disabilities cost-efficient to serve via supported employment programs?

The relations between cost-efficiency (from the perspective of worker, taxpayer, and society) and personal characteristics of supported employees (i.e., IQ, level of mental retardation, multiple disabilities, gender, ethnicity, and age) were examined. Results suggest that when sheltered workshops were used as alternative placements, supported employees with high IQs benefited more from employment within the community than did supported employees with lower IQs. From society's perspective, African American and male supported employees were more cost-efficient than were European American and female supported employees. Further, regardless of the severity or number of disabilities, all individuals were cost-efficient from each perspective (i.e., worker, taxpayer, and society) and time period (i.e., 1990, 1994, and projected lifelong).     

Incidence of excessive/impacted cerumen in individuals with mental retardation: a longitudinal investigation

A major sequelae of impacted cerumen (earwax) is conductive hearing loss, which can be deleterious to linguistic, social, and intellectual development, particularly for individuals with mental retardation. The longitudinal incidence (12-year period) of excessive/impacted cerumen and related conductive hearing loss of 117 adults with mental retardation was examined. Results indicated a considerably higher incidence of excessive cerumen and cerumen impaction with associated conductive hearing loss in adults with mental retardation (28%) than in the general adult population (2% to 6%). Moreover, a strong propensity for recurrent cerumen impaction in the mentally retarded population was found. These findings were discussed in view of the management of cerumen impaction among individuals with mental retardation.     

Validity of the PAS-ADD for detecting psychiatric symptoms in adults with learning disability (mental retardation)

The Psychiatric Assessment Schedule for Adults with Developmental Disability (PAS-ADD) is a semi-structured interview for use with respondents who have learning disability and for key informants. This report investigates the ability of the instrument to detect symptoms that had been found to exist during routine clinical assessment of the patients. Field trials involved 95 referred patients with learning disability and a key informant for each sample member. Clinical opinions of the referring psychiatrists were sought using a symptom checklist. Referrer checklist symptoms and PAS-ADD data were both factor analysed. Validity testing involved (a) computation of correlations between PAS-ADD factors and checklist data and (b) comparison of PAS-ADD and referrers' diagnoses. Results indicated good validity for the PAS-ADD in relation to psychotic symptoms and depressive symptoms. Anxiety symptom identification was not well validated, probably due to small numbers. Expansive mood identified by the referrers was not detected by the PAS-ADD because there is currently no corresponding section in the interview. Where the PAS-ADD produced a diagnosis (in 58 members of the sample), 44 were in agreement with the referrer. Probability of diagnosis by PAS-ADD increased with the number of relevant active symptoms identified by the referrer. The PAS-ADD has been shown in a previous report to have the sensitivity to detect mental disorders not known to psychiatric services. For psychotic and depressive conditions, our results showed that symptom detection was in good agreement with the information provided by the referring psychiatrists on their patients. The PAS-ADD needs a section on hypomania and further investigation of its detection of anxiety disorders.     

Akathisia in adults with mental retardation: development of the Akathisia Ratings of Movement Scale (ARMS)

We developed an akathisia rating scale for use with persons who have mental retardation and screened for the occurrence of akathisia in three samples: 66 adults receiving maintenance neuroleptic treatment, 20 adults not receiving neuroleptics, and 8 adults undergoing neuroleptic dose reduction. The scale had an acceptable level of interrater reliability and validly measured group differences related to neuroleptic treatment status. Using an empirically derived cut-off-score, we estimated the prevalence rate for akathisia to be 5% in neuroleptic-free subjects, 17% in neuroleptic-maintenance subjects, and 25% in neuroleptic-reduction subjects. Akathisia, dyskinesia, and stereotypy manifested as qualitatively different movement topograhies. The occurrence of dyskinesia stereotyped movement disorder was associated significantly with an increased occurrence of akathisia.     

Comparison of opportunities to respond within a drill model when rehearsing sight words with a child with mental retardation.

Sight-word instruction can improve functioning with various daily, recreational, and work-related tasks among children with moderate to severe disabilities. Previous research has demonstrated the effectiveness of drill methods to teach sight words if the model contains at least 50% known items, which would also increase the number of opportunities to respond (OTR) to the target item. The current study taught five sight words each day to a child diagnosed as moderately mentally retarded using high OTR and moderate OTR conditions and a drill ratio of 10% unknown to 90% known. Results suggested that the high OTR condition led to better retention than the moderate OTR despite containing an equal ratio of unknown to known items between them. Implications for practicing school psychologists and future research are included. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Implicit and explicit memory in individuals with mental retardation

Students with and without mental retardation from three age groups were compared on implicit and explicit memory tasks. Consistent with previous research on intelligence-related differences in controlled and automatic processes, students without mental retardation performed better than those with mental retardation on the explicit memory task, but there was no difference between groups on the implicit memory task. For both groups implicit and explicit memory increased from age 6 to 8 to age 10 to 12, but did not significantly increase to age 15 to 17. Because implicit memory appears to be a relative strength for students with mental retardation, we suggest further exploration into broader types of implicit processes that may be useful in training situations.     

Interrater agreement and stability of the Functional Independence Measure for Children (WeeFIM): use in children with developmental disabilities

OBJECTIVE: Examination of the interrater agreement and stability of ratings obtained using the Functional Independence Measure for Children (WeeFIM) in a sample of children with developmental disabilities. DESIGN: A relational design was used in which two sets of WeeFIM scores were collected under four conditions: same rater-short interval; same rater-long interval; different rater-short interval; and different rater-long interval. SETTING: WeeFIM scores were collected in outpatient developmental rehabilitation centers, school programs, and the children's homes. PARTICIPANTS: Data were collected for 205 children ranging in age from 11 to 87 months. All children had a medical diagnosis of disability and were receiving habilitative-educational intervention or follow-along services including neurodevelopmental surveillance. INSTRUMENT: The WeeFIM instrument examines basic daily living and functional skills in children from birth to 7 years of age. The WeeFIM is modeled after the Functional Independence Measure (FIM) for adults and includes 18 items in the following subscales: self-care, sphincter control, transfers, locomotion, communication, and social cognition. RESULTS: Kappa values for items ranged from .44 to .82. Intraclass correlation coefficients (ICC) for the six subscales ranged from .73 to .98. Total WeeFIM ICC values were greater than .95 for all analyses. CONCLUSIONS: The WeeFIM ratings for the 205 children with developmental disabilities participating in this investigation were consistent across raters and time.     

Mental illness and mental retardation: Message from the President of the United States relative to mental illness and mental retardation.

2 health problems of critical size and tragic impact are mental illness and mental retardation. "There are now about 800,000 such patients in this Nation's institutions—600,000 for mental illness and over 200,000 for mental retardation." A 3-fold attack is proposed: (a) Ascertain causes and eradicate them. (b) Strengthen underlying resources of knowledge and of skilled manpower. (c) Strengthen and improve facilities serving the mentally ill and mentally retarded. A national program for mental health is proposed which emphasizes comprehensive community mental health centers, improved care in state mental institutions, and expansion of research activities and increase in professional manpower. A national program to combat mental retardation emphasizing prevention, community services, and research is also proposed. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Primary care of adults with mental retardation living in the community

An increased number of adults with mental retardation are living in the community and seeking health care from family physicians. When mentally retarded patients are enrolled in a medical practice, guardianship status should be determined, but these patients should be involved in their own care to as great an extent possible. Since a verbal history may be difficult to obtain, a systematic, thorough physical examination is important. Certain Illnesses, such as hepatitis B, recurrent aspiration syndrome, leukemia and atlantoaxial instability, are much more common in adults with Down syndrome then in adults with mental retardation from other causes. Seizures and mental illness are equally common in all mentally retarded adults. The behavior management and pharmacologic therapy of patients with mental retardation are best handled in close association with caregivers, as well as psychiatrists and neurologists who are familiar with the special needs of this population.     

Predicting the earnings of supported employees with mental retardation: a longitudinal study

A random sample (N = 197) of supported employees with mental retardation was examined in a longitudinal study. Results indicate that intelligence, prior earnings, and federal job subsidy predicted future earnings. Statistical controls applied to the stratified sample show that job placement, job type, subsidy, and means of transportation had little influence on earnings.     

Prevalence of mental retardation in patients with hereditary retinoblastoma

Transvaginal amniotic puncture (TAP) was performed on 20 consecutive missed abortions immediately prior to dilatation and evacuation and the cytogenetic results compared. The information received from products of conception (POC) and TAP was in concordance in only 5 of 20 (25%) cases. Tissue obtained from POC yielded cells in all instances. However, only 3 of 20 POC samples yielded findings other than normal female. In contrast, 92.8% of the conclusive diagnoses would have been achieved by TAP alone. These data strongly suggest that TAP is superior to POC for accurate cytogenetic assessment of missed abortion and should lead to a reevaluation of our current understanding and management of pregnancy loss.     

Four sibs with dislocated elbows, bowed tibiae, scoliosis, deafness, cataract, microcephaly, and mental retardation: a new MCA/MR syndrome

We report four sibs with an MCA/MR syndrome whose parents were first cousins. The sibs had mental retardation, microcephaly, hearing problems, cataract, and multiple osseous malformations, such as dislocated elbows, bowed tibiae, and scoliosis. Review of published reports and the use of the London Dysmorphology Database suggest that this family presents a new syndrome.     

The inv dup(15) syndrome: a clinically recognizable syndrome with altered behavior, mental retardation, and epilepsy

The most common of the heterogeneous group of the extra structurally abnormal chromosomes (ESACs) is the inv dup(15), whose presence results in tetrasomy 15p and partial tetrasomy 15q. Inv dup(15), containing the Prader-Willi/Angelman syndrome (PWS/AS) region, are constantly associated with phenotypic abnormalities and mental retardation. We report on four additional patients with inv dup(15), whose behavioral pattern, and neurologic and physical findings further delineate the phenotype of this neurogenetic syndrome. We also provide FISH analyses on chromosomes of the observed ESACs and discuss the role of a number of genes located within the tetrasomic region.     

Hemorrhagic manifestation of moyamoya disease in a 19-year-old woman with mental retardation

About 85% of all births in the US occur to women under 35 years of age. According to the US National Survey of Family Growth, women aged 35-39 years expect to have an additional 0.3 births while those 40-44 years anticipate only 0.1 additional births. Thus, there is a need to protect women age 35 years and over from unwanted pregnancy until menopause occurs. Sterilization continues to be the most common choice for married women older than 30 years. The IUD may be an excellent alternative to sterilization. Oral contraceptive (OC) use declines in women over 35 years because of concerns about increased cardiovascular risk. However, new studies have indicated myocardial risk in older women is mainly limited to women with other cardiovascular risk factors such as smoking, hypertension, and diabetes. Moreover, the OC-related benefits of protection against ovarian and endometrial cancer, reduced menstrual blood loss, and increased bone density are especially important to women over 35 years. Since it is impossible to diagnose menopause within the pill-free week, OC users should stop the pill for a month at around 50 years of age to allow accurate follicle-stimulating hormone measurement.     

Epidemiology and prevalence of psychopathology in people with mental retardation.

There is a general consensus that, for several reasons, people with mental retardation are at an increased risk of developing emotional disorders. Numerous research studies have examined the prevalence of psychiatric disorders among people with mental retardation, and a wide range of rates have been reported. Reasons for the variability in these results are discussed, including definitional and identification issues, and sampling issues. A summary of representative studies is presented and discussed in light of the above-mentioned issues. The need for updated epidemiological studies in this area is emphasized. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Competence and adjustment of siblings of children with mental retardation

Adjustment and competence of siblings who had a brother or sister with mental retardation were compared to those of control siblings who had brothers or sisters with no disabilities. Results indicated no overall differences for internalizing disorders, externalizing disorders, or for self-esteem and competence based on group membership, gender, or gender match. However, boys with a brother or sister with mental retardation had difficulty in school functioning. In addition, a greater number of girls with brothers and sisters with mental retardation expressed their distress through internalization.