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Mental retardation and the acquisition of automatic processing.

The acquisition of automatic processing in persons with and without mental retardation was examined. In Experiment 1, subjects viewed slides of objects to determine whether a pictured object belonged to a designated target category. There was an effect of search set size for both groups that decreased and eventually disappeared with practice. This result reflected the acquisition of automatic processing. Also, evidence of automaticity was observed with less practice for subjects without relative to subjects with mental retardation. In Experiment 2, subjects searched for the presence of a designated target shape in arrays containing two, three, or four shapes. Results were essentially the same. Implications of these results for the development of cognitive skills by persons with mental retardation were discussed.

Adolescent↗

A Rett syndrome MECP2 mutation that causes mental retardation in men.

OBJECTIVE: To characterize the clinical features of a new type of X-linked mental retardation associated with MECP2 mutation in the index family. BACKGROUND: MECP2 mutations, originally described in a high percentage of patients with classic Rett syndrome, were considered lethal in men. The authors recently described a novel A140V MECP2 missense mutation in an Italian family with X-linked semidominant mental retardation. METHODS: The neurologic features of six symptomatic relatives (two women and four men) carrying the mutation were compiled. Laboratory investigations included EEG, EMG, conduction velocity (CV) of peripheral nerves, brain MRI, and (1)H-MR spectroscopy. RESULTS: Mental retardation and signs of neurologic impairment were present in all the affected members, but more pronounced in men. Neurologic features included slowly progressive spastic paraparesis/pyramidal signs (6/6), distal atrophy of the legs (6/6), ataxia (2/6), and postural tremor of the hands (3/6). Speech was preserved (6/6) but was dysarthric in the oldest brothers (2/6). Mild dysmorphic features were present in all cases. CONCLUSION: The neurologic disorder associated with A140V MECP2 mutation is not necessarily lethal in men, but they are more severely affected than women of the same family.

Adult↗

A cytogenetic survey of mentally retarded children in Taiwan: final report on the incidence of chromosome abnormalities.

A cytogenetic survey of 470 mentally retarded children from three different sources is reported. Thirty-eight patients (8.08%) were found to have recognizable chromosome abnormalities, including 4 cases of sex chromosomes and 34 cases of autosomes. The most prominent category of abnormality was trisomic 21, a total of 26 cases of this type was found in this study. Sex chromosome abnormalities contribute very little to the etiology of mental retardation. From the results of our three groups of patients as well as from those surveyed by others, it is concluded that the more severe the degree of mental retardation, the higher the incidence of chromosome abnormalities.

Adolescent↗

Probabilities of the development of ambulation for mobile children with moderate to profound mental retardation.

Development of ambulation was studied among 1,744 children, ages 3 through 10 years, with moderate to profound mental retardation. The sample was limited to children with some degree of mobility at initial testing. Evaluations over 6 years indicated that improvements in basic skills, although not probable, do occur far past the normative age for development of speech and ambulation and across all levels of mental retardation. The probability of improvement declined with age. Degree of mental retardation and severity of motor impairment had the expected negative effects on initial skill level and probability of improvement. Limiting the sample to subjects who were mobile minimized the effect of mortality.

Age Factors↗

Diagnosis and treatment of mental illness in mentally retarded children: a developmental model.

Attempts have been made in recent years to discover the roots of psychiatric disorders in mentally retarded children by employing a developmental approach in which the child, not the handicap, is brought more clearly into focus. This paper provides a brief overview of the developmental model that has proven useful for the author in the psychiatric diagnosis of mentally retarded children. Application of this model to the treatment of mentally ill-mentally retarded children is also addressed.

Child↗

Habituation of optically evoked blink reflex in mentally retarded adults.

This study was done to record the difference in habituation patterns of optically evoked blink reflexes in mentally retarded and normal adults. Sets of photic stimuli with fixed interstimulus duration were utilized in normal volunteers and mentally retarded adults. The interstimulus duration decreased with each sequential set. The blink responses were recorded on fibreoptic recording paper. These electrophysiological methods show objectively that the blink responses to light which habituate in normal adults fail to do so in those who are mentally retarded.

Adult↗

Who is interested in careers in mental retardation and developmental disabilities?

In an effort to understand factors that may influence individuals to pursue careers in mental retardation, we asked students enrolled in a 10-week course and practicum experience to complete a pretest-posttest assessment of career interest. Results indicated that students having prior exposure to individuals with mental retardation reported significantly higher levels of career interest than did students without such experience. There were no significant changes in career interest from pre- to posttest. Students with a history of volunteering with individuals who had mental retardation comprised the largest group of students reporting career interest.

Adolescent↗

Laparoscopic Thal fundoplication in mentally retarded children.

BACKGROUND: An increasing number of reports indicate that Thal fundoplication is the procedure of choice in mentally retarded children. With the advent of laparoscopy, Nissen's fundoplication seems to have been repopularized. However, the choice of the operative technique should be based on the merits of the procedure itself rather than the laparoscopic feasibility. The aim of this study is to determine if laparoscopic Thal fundoplication is beneficial for mentally retarded children. METHODS: Between November 1993 and 1994 laparoscopic Thal fundoplication was performed in 15 mentally handicapped children; 13 also had a feeding gastrostomy. Age varied from 1.5 to almost 17 years (mean 7 years). Mean weight was 18 kg (5-50 kg). All patients underwent an upper GI study and endoscopy as well as pre- and 3 months postoperative pH study. Indications for the procedure were reflux esophagitis in 11 and feeding problems with silent reflux in 4. RESULTS: The laparoscopic procedure was converted in the second patient because of bleeding in the hiatus. No further procedure-related intra- or postoperative complications occurred. The mean hospitalization was 3.7 days. No symptomatic postoperative gastroesophageal reflux has been observed. All children have undergone postoperative pH studies, which displayed silent reflux in two. Gastrostomy feeding is well tolerated. CONCLUSION: We conclude that Thal fundoplication can be performed laparoscopically in mentally retarded children. The laparoscopic results are comparable to the open Thal procedure.

Aged↗

Some characteristics of a population of mentally retarded young adults in a British city. A basis for estimating some service needs.

A population of young people who are mentally retarded is described in terms of the frequency of various kinds and degrees of impairment--intellectual, physical and behavioural. The population consisted of all those born in 1951--1955 who were resident in a city in 1962 and who at any time during the school years had been placed in any mental retardation service. The prevalence of the mentally retarded as defined is sixteen per 1000. Age specific prevalence rates for the same population rise from age five, reach a plateau of fourteen per 1000 from ages ten to fifteen and then drop sharply for the post-school period to a rate of five to six per 1000. Classifications of physical impairment, seizure histories and behaviour disturbances are described. Percentages are given separately for those with each impairment and in combination for each individual. For the survivors at age twenty-two, 44 per cent had physical impairments, 26 per cent had a history of seizures and 46 per cent had experienced behaviour disturbances. Results are discussed in relation to service needs.

Adolescent↗

Depression and the onset of dementia in adults with mental retardation.

The relation between dementia and depression in 61 adults with Down syndrome or 43 adults with mental retardation due to other causes was examined. Age-matched participants, ranging in age from 20 to 60 years, received a neuropsychological battery to assess declines in functioning and caregiver report measures to assess adaptive behavior and depression. Eight adults with Down syndrome had both depression and declines in functioning. No adults with mental retardation due to other causes had declines. Greater severity of depression was related to lower MA, poorer memory, and lower adaptive functioning in adults with Down syndrome only. Results suggest that dementia and depression are associated in Down syndrome but not in mental retardation due to other causes.

Activities of Daily Living↗

Non-skewed X-inactivation may cause mental retardation in a female carrier of X-linked alpha-thalassemia/mental retardation syndrome (ATR-X): X-inactivation study of nine female carriers of ATR-X.

X-linked alpha-thalassemia/mental retardation syndrome (ATR-X) is a syndromic form of X-linked mental retardation. We investigated the X-inactivation status of nine female ATR-X carriers by methylation-specific PCR of the HUMARA gene. Six carriers demonstrated a skewed X-inactivation pattern (>90:10) and one showed a non-skewed pattern (72:28), while two were uninformative because of homozygosity for the CAG repeat polymorphic alleles in the HUMARA. Only the carrier mother who showed non-skewed X-inactivation had moderate mental retardation. These findings suggest that mutations in ATRX may cause mental retardation in females, if the X chromosome carrying mutated ATRX is not properly inactivated.

Chromosomes, Human, X↗

Measuring problem behaviors in children with mental retardation: dimensions and predictors.

Scores from the Child Behavior Checklist (CBCL; Achenbach, 1991a) and the Client Development Evaluation Report (CDER; California Department of Developmental Services, 1980) for 67 children and adolescents with mental retardation were examined to evaluate the factorial validity of the instruments. Four factor analyses were conducted. The initial factor analysis of CBCL data failed to confirm the presence of the five first-order factors previously reported for the CBCL standardization sample (Achenbach, 1991b). Second, the higher-order factors of Externalizing and Internalizing behaviors, similar to the structure reported for the CBCL standardization sample (Achenbach, 1991b), were confirmed on the present sample. Third, the two CDER factors of Personal Maladaption and Social Maladaption, previously identified by Widaman, Gibbs, and Geary (1987), were also confirmed. Finally, a higher-order factor analysis of the two factor scores from the CBCL and two factor scores from the CDER was conducted to study the congruence between the CBCL Externalizing and CDER Social Maladaption dimensions, and between the CBCL Internalizing and CDER Personal Maladaption factors. Moderate levels of congruence were found. Next, child characteristics, including level of mental retardation, age, and four dimensions of adaptive behavior, were used as predictors of problem behavior. No child characteristics were significantly related to the CBCL Externalizing dimension, but child age and level of mental retardation were significant predictors of the CBCL Internalizing dimension. CDER Cognitive Competence predicted CDER Social Maladaption, and child age predicted CDER Personal Maladaption. The findings are discussed in relation to previous studies of problem behaviors of children and adolescents with mental retardation.

Adolescent↗

Mental retardation, spasticity, and transverse limb defects.

We report on a mentally retarded boy with spastic paraplegia and a transverse defect of the left foot who was born to consanguineous parents. This association of ectrodactyly, mental retardation, and spastic paraplegia was first reported by Jancar [1967].

Abnormalities, Multiple↗

[Mental retardation in children as a risk factor in pregnancy].

The mentally retarded child is a risk factor of the subsequent pregnancy. This risk is usually not taken into account. Of 205 mothers with a mentally retarded child 95 became again pregnant. In 15 instances they gave birth to an affected child. During the preconception period almost one third did not attend any medical examination. With the problem of prenatal diagnosis interfere among others also socio-economic factors and sometimes also a low activity of health workers.

Adolescent↗

Hepatitis B control in Toronto classrooms for the mentally retarded: a seroprevalence survey.

A seroepidemiologic hepatitis B survey of students and staff at schools for the mentally retarded in the City of Toronto found 2.5% of students to be carriers, 5% to be immune without being immunized, 11.3% to have immunization underway or complete, and 81.2% to be susceptible. The first two groups were older and more likely to have been born in countries with intermediate or high hepatitis B marker prevalence. Among staff tested, 4.1% were immune without immunization while all others were susceptible. Because hepatitis B control recommendations could not be made on the basis of these results alone, local Medical Officers of Health also considered other studies and practical experience to recommend the following: (1) Use Ontario Ministry of Health Guidelines for mentally retarded hepatitis B carriers in regular schools; (2) Offer hepatitis B vaccine to all susceptible students and staff in schools specifically for the mentally retarded.

Carrier State↗

A new case of Balci's syndrome (corneal opacity, microphthalmia, microcephaly, mental retardation, and generalized muscular spasticity associated with congenital heart disease).

The association of corneal opacity, microphthalmia, microcephaly, mental retardation, and generalized muscular spasticity with hyperglycinemia was presented for the first time by Balci and colleagues in 1974. After this report, some similar cases in the literature were referred to as Balci's syndrome. In this paper we describe a new case of Balci's syndrome, a 2.5-month-old female patient with corneal opacity, microphthalmia, microcephaly, mental retardation, and generalized muscular spacticity. All of these findings are acceptable as Balci's syndrome, and in addition she had congenital heart disease (ventricular septal defect) and renal anomalies. In this paper other syndromes associated with corneal opacity and mental retardation are discussed.

Abnormalities, Multiple↗

Thermodynamics of the fragile X mental retardation protein RGG box interactions with G quartet forming RNA.

Fragile X syndrome, the most common form of inherited mental retardation, is the result of an unstable expansion of a CGG trinucleotide repeat in the 5' UTR of the fragile X mental retardation-1 (FMR1) gene. The abnormal hypermethylation of the expanded CGG repeats causes the transcriptional silencing of the FMR1 gene and, consequently, the loss of the fragile X mental retardation protein (FMRP). FMRP is an RNA binding protein that binds to G quartet forming RNA using its RGG box motif. In this study we have performed a thermodynamic analysis of the interactions between the FMRP RGG box domain and Sc1, an RNA molecule which had been previously shown to be bound with high affinity by both the full-length FMRP and by its RGG box domain. We have determined that the association between the FMRP RGG box and Sc1 RNA is dominated by hydrophobic and hydrogen bond interactions, with minor contributions from electrostatic interactions, and that the FMRP RGG box binding increases the stability of the G quartet RNA structure significantly. Interestingly, we found that the G quartet recognition is necessary but not sufficient for the FMRP RGG box binding to this RNA target, indicating that additional interactions of the peptide, possibly with the stem and/or stem-G quartet junction region, are required. Our results also indicate that the G quartet RNA recognition is not a general feature of the RGG box motif but rather carries some sequence, protein and/or RNA, specificity.

Amino Acid Sequence↗

Relationship between age and head movement during stepping in place by nonhandicapped children and persons with mental retardation.

We investigated developmental changes in head movement during stepping in place by nonhandicapped children and clarified the relationship between chronological or mental age and head movement of persons with mental retardation. 39 nonhandicapped children aged 3 to 6 years, 13 adults aged 19 to 22 years, and 10 persons with mental retardation (range of chronological age: 17 to 22 years, range of mental age: 3 to 8 years) participated. The total extent of head movement in the frontal plane during ordinary stepping in place for 20.48 sec. was measured to assess the magnitude of head movement. Changes in the magnitude of head movement of children are noticeable across ages, and the magnitude clearly was smaller at the age of 6 years. For subjects with mental retardation, no relationship has been found between chronological age and the magnitude of head movement but rather between mental age and the magnitude of head movement.

Adult↗