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Biomedical subjects

H M Evenhuis

Publications and source records attributed to H M Evenhuis.

At least 19 recordsLinked to original sources
PubMed2005

Prevalence of visual impairment in adults with intellectual disabilities in the Netherlands: cross-sectional study.

PURPOSE: To obtain the first representative and valid population-based prevalence figures on visual impairment and blindness in adults with intellectual disabilities (ID) and to identify risk groups. METHODS STUDY DESIGN: Cross-sectional survey. An age-Down's syndrome-stratified random sample of 1,598 persons from a base population of 9,012 adult users of ID services with mild to profound intellectual disabilities was screened. Participants underwent protocollised on-site screening of visual functions. Results were related to degree of ID, occurrence of Down's syndrome (DS) and age. MAIN OUTCOME MEASURE: Prevalences of visual impairment and blindness in the study population and in subgroups and weighted prevalences in the total Dutch population using ID services. RESULTS: Prevalences of visual impairment ranged from 2.2% (95% confidence interval (CI), 0.5-6.4) in young adults with mild ID and no Down's syndrome to 66.7% (95% CI, 41.0-86.7) in older adults with profound ID and Down's syndrome; prevalences of blindness ranged from 0.7% (95% CI, 0.1-4.1) to 38.9% (95% CI, 28.1-50.3). Weighted prevalences of visual impairment and blindness in the total Dutch population of adult users of intellectual disability services are 13.8% (95% CI, 9.3-18.4) and 5.0% (95% CI, 3.8-6.2), respectively. Prior to this study, visual impairment or blindness had remained undiagnosed in 106/261 (40.6%) persons. CONCLUSIONS: As compared to published figures for the general Dutch population aged 55 years and over (visual impairment 1.4%, blindness 0.5%), prevalences of visual impairment and blindness are higher in all subgroups with intellectual disabilities, including the young and mildly handicapped group. The diagnosis is too often missed. All persons with severe or profound intellectual disabilities, and all older adults with Down's syndrome, should be considered visually impaired until proved otherwise.

Adult↗
PubMed2003

Visual performance in specific syndromes associated with intellectual disability.

PURPOSE: To report visual performance in adults with specific causes of intellectual disability (ID) and to compare the test results to published reports. METHODS: In a large-scale multicenter epidemiologic study of sensory impairments in 1598 adults with ID, the authors performed ocular assessments in 1539 persons. They compared the test results of those with five specific genetic disorders (Angelman syndrome, Prader-Willi syndrome, fragile X syndrome, Williams-Beuren syndrome, and tuberous sclerosis). RESULTS: An overrepresentation of strabismus, low vision, and refractive errors was found. Apart from fragile X syndrome and Prader-Willi syndrome (with in general mild to moderate ID), the other syndrome groups contained one or more subjects with visual impairment or blindness. A number of them had never been seen by an ophthalmologist. CONCLUSIONS: The authors confirm a number of ocular features previously reported by other studies and suggest some additional ocular features. They found increased frequencies of treatable ophthalmologic conditions in the subgroups. Because reliable ocular assessment is feasible for 85% of persons with ID, the results are an incentive to address visual functioning in people with ID in order to correct ocular problems and maximize their possibilities.

Abnormalities, Multiple↗
PubMed2001

Prevalence of visual and hearing impairment in a Dutch institutionalized population with intellectual disability.

A screening of hearing and visual function was performed using clinical assessment methods in a Dutch institutionalized population of 672 people with mild to profound intellectual disability (ID). Because the studied population was not comparable to the total Dutch population with ID, subgroups were distinguished according to level of ID, age younger and older than 50 years, and the presence or absence of Down's syndrome (DS). The prevalences of both hearing and visual impairment were considerably increased in all subgroups, as compared with the general population. In the least affected group, i.e. those < 50 years with a mild or moderate ID by other causes than DS, the prevalences of hearing and visual impairment were 21% and 4%, respectively (as opposed to 2-7% and 0.2-1.9% in the general Dutch population <50 years, respectively). The prevalence of hearing impairment showed a sharp and highly significant increase in individuals with DS and subjects > or = 50 years. To a lesser extent, young adults with severe or profound ID had an increased risk of hearing impairment. Visual impairment and blindness were specifically highly prevalent in people with severe or profound ID (51% < 50 years of age). Down's syndrome and an age > or = 50 years were also significant risk factors for visual impairment. There was an alarmingly high prevalence of combined sensory impairment, especially in those with severe or profound ID (20%). Although hearing impairment had been diagnosed prior to this screen in 138 people and visual impairment in 65 individuals, a first diagnosis of hearing impairment was made in 128 subjects and of visual impairment in 90 cases. This highlights the tendency for sensory impairments to go unnoticed in people with ID, which is not restricted to those with severe or profound ID. Therefore, the present authors stress the importance of regular screening as outlined in the existing IASSID international consensus statement.

Adolescent↗
PubMed1999

[Practice guidelines for diagnosis of vision disorders in mentally handicapped persons. National Organization for Quality Assurance in Hospitals].

Visual impairment is more frequent among people with intellectual disability than among the general population. Because the diagnosis often fails to be made in this group screening is justified. Timely recognition may have consequences for prevention and treatment. Therefore, a consensus statement has been developed by the Dutch associations of physicians for intellectual disability, general practitioners, ophthalmologists, paediatricians, youth health physicians and orthoptists. Specialized ophthalmological assessment of all young children with retarded development is recommended, as well as assessment of visual function at the ages of 3, 6, 12 and 18 years and every 5 years from the age of 45. In adults with Down's syndrome, an additional assessment at the age of 30 is recommended for detection of age-related cataract and (increased) refractive errors. Most people can be assessed by the general practitioner, using normal methods. Diagnostic methods applicable in insufficiently co-operative people have been recommended. Low-threshold regional expert centres for diagnosis in children and adults who are difficult to assess and for specialized guidance would be advisable.

Adolescent↗
PubMed1997

Medical aspects of ageing in a population with intellectual disability: III. Mobility, internal conditions and cancer.

The aim of this study was to assess mobility impairment and morbidity and mortality caused by internal conditions and cancer in an ageing population with mild to severe intellectual disability. Seventy subjects with a mean age of 70 years (range 60-92 years) at initial evaluation, were assessed during a 10-year prospective longitudinal study. As was to be expected, there was a high prevalence of mobility impairment (30% under and 58% over age 75), similar to reports from other ageing populations with intellectual disability, and of related conditions (chronic constipation, pulmonary function loss and urinary incontinence). Overall morbidity and mortality caused by internal disease and cancer might be comparable to that in the general ageing Dutch population, but questions concerning the specific contribution of risk factors will have to be addressed. The lack of or atypical presentation of subjective symptoms was striking. Nevertheless, autopsy outcomes showed that diagnosis in people with intellectual disability can be as accurate as in the general population, if performed actively.

Aged↗
PubMed1997

The natural history of dementia in ageing people with intellectual disability.

In a retrospective follow-up study over 11 years, the incidence and natural history of dementia was assessed in an institutionalized group of 144 people, aged 60 years and over, with mild to profound intellectual disability resulting from causes other than Down's syndrome. Age-related incidences, age at onset, duration and symptoms of dementia were comparable to those in the general population. The frequent and invalidating physical comorbidity (11/11) hampered the diagnostic process in this group. The high prevalence of episodes of delirium (9/11) and depressive symptoms (8/11) as the first manifestations of dementia and/or during dementia might reflect increased vulnerability as compared to other ageing people.

Aged↗
PubMed1997

Diagnosis of sensory impairment in people with intellectual disability in general practice.

The present authors have participated in the development of a Dutch consensus on the early detection, diagnosis and treatment of hearing and visual impairment in children and adults with intellectual disability. They argue that the early detection of sensory impairment in babies and children with intellectual disability should primarily be a responsibility of paediatricians and youth health physicians. General practitioners should be aware of the necessity of screening and should check whether this has been done when children visit the surgery. It is stressed that the general practitioner should play a more active role in the detection of age-related sensory loss in older adults with intellectual disability, and the assessment of younger adults whose sensory functions have never or incompletely been evaluated. Annual sensory screening is certainly not necessary, but annual otoscopy to detect impacted earwax or unidentified middle ear infection, as well as checks of the proper use of glasses and hearing aids, are suggested. Most adults with mild or moderate intellectual disability can be assessed with methods that are normally used by general practitioners. Uncooperative people should be referred for screening with specialized methods. A low-threshold referral system (e.g. via district expert teams) has been outlined.

Adolescent↗
PubMed1996

[Cancer incidence in persons with a mental handicap; possibly increased risk of esophageal cancer].

OBJECTIVE: Prompted by a British study which revealed a raised frequency of oesophageal cancer as the cause of death among the mentally handicapped, a study was made of the cancer incidence among an institutionalized population with a mental handicap, which incidence was compared with that among the population in general. DESIGN: Retrospective follow-up study. METHODS: An inventory was made of the cytologically or histologically confirmed cancer diagnoses among persons living in an institute for the mentally handicapped during 1 January 1974 through 1 January 1994 (n = 1020). The incidence figures were compared with those for the general population (Netherlands Cancer Registration 1989) after indirect standardization. RESULTS: The total cancer risk among the population with a mental handicap was the same as that among the general population where women, and lower where men were concerned. This study brought to light a decreased risk of lung cancer (standardized morbidity rate (SMR): 0.33; 95% confidence interval: 0.33-0.97), while the SMR of oesophageal cancer showed no statistically significant increase. CONCLUSIONS: Cancer is not more frequent among the mentally handicapped than among the general population. Nevertheless, increased attention for occurrence of oesophageal cancer appears to be still indicated.

Adolescent↗
PubMed1996

Further evaluation of the Dementia Questionnaire for Persons with Mental Retardation (DMR).

The provisional diagnostic criteria for the Dementia Questionnaire for Persons with Mental Retardation (DMR), developed during a prior study, were evaluated in a 5-year longitudinal follow-up of 33 elderly institutionalized persons, aged 70 years and over, and 45 institutionalized persons with Down's syndrome, aged 35 years and over, with no dementia in the diagnosis at initial evaluation. During the study period, dementia was diagnosed according to DSM-III-R criteria in five elderly subjects and five subjects with Down's syndrome, whereas a diagnosis of possible dementia was made in two elderly subjects and three subjects with Down's syndrome. A DMR diagnosis based on the criterion increase over time of the sum of cognitive scores (SCS) > or = 7 points and/or of the sum of social scores (SOS) > or = 5 points' resulted in a sensitivity of 100% for both groups and a specificity of 73% in the elderly sub-group and 75% in the sub-group with Down's syndrome, independent of the (premorbid) intellectual level. A diagnosis based on a single completion of the DMR, using available information on former performance levels, also produced favourable results in the present study. However, this diagnostic approach is certainly not recommended for studies of larger samples because of the use of different methods measuring functional levels and different standards for levels of intellectual disability.

Aged↗
PubMed1996

Dutch consensus on diagnosis and treatment of hearing impairment in children and adults with intellectual disability. The Consensus Committee.

This is an abridged translation of a recent Dutch consensus on diagnosis and treatment of hearing impairment in children and adults with intellectual disability. Available diagnostic methods are discussed. The use of Oto-Acoustic Emissions as a rapid and low-cost objective screening method is particularly encouraged. Risk groups, protocols for early identification and diagnostic screening in older children and adults, recommendations for treatment and support, and priorities for research have been formulated.

Acoustic Impedance Tests↗
PubMed1995

A case-control study of apolipoprotein E genotypes in Alzheimer's disease associated with Down's syndrome. Dutch Study Group on Down's Syndrome and Ageing.

The prevalence of clinical signs and neuropathological findings of Alzheimer's disease (AD) is high in Down's syndrome (DS). In the general population, the apolipoprotein E (ApoE) epsilon 4 isoform is an important risk for AD. We studied the allelic frequencies of ApoE in 26 DS cases fulfilling clinical diagnostic criteria for AD and in 26 DS controls matched for age, sex, and premorbid level of mental retardation. A meta-analysis of data available in the literature was used for comparison with allele frequencies in other AD and control populations. ApoE type 2, 3, or 4 allele frequencies were not significantly different in AD-DS cases and DS controls. The ApoE epsilon 4 frequency in DS cases with AD (0.14; CI, 0.06-0.26) was significantly lower than in any other AD population studied so far and it is within the range of nondemented controls from the general population. These findings suggest that ApoE epsilon 4 does not significantly affect the pathogenesis of AD in DS patients.

Adult↗
PubMed1995

Medical aspects of ageing in a population with intellectual disability: I. Visual impairment.

Visual function of an institutionalized population with intellectual disability, consisting of 70 subjects with a mean age of 70.1 (range 60-92) years at initial evaluation, was assessed during a 10-year prospective longitudinal study. One subject had Down's syndrome and could not be assessed as a result of dementia. Lower visual acuity values were relatively overrepresented as compared to reported data from ageing populations without intellectual disability. In addition, the prevalence of moderate to severe visual impairment was distinctly higher (27.9% in the group studied vs. 0.66% at age 60-69 years to 13% over age 80 in a population without intellectual disability). During follow-up, visual function improved in three out of 61 subjects (4.9%) after cataract surgery, and deteriorated in eight out of 61 subjects (13.1%), even with optimal correction, as a result of cataract and macular degeneration. Causes of excess impairment were congenital or childhood conditions, too-late diagnosis of glaucoma and suboptimal correction of refractive errors in non-cooperative individuals. The present author concludes that it should be possible to reduce excess impairment by an active diagnostic and therapeutic attitude to subjects from a young age onwards.

Age Factors↗
PubMed1995

Medical aspects of ageing in a population with intellectual disability: II. Hearing impairment.

Hearing function of an institutionalized population with intellectual disability, consisting of 70 subjects with a mean age of 70.1 (range 60-92) years at initial evaluation, was assessed during a 10-year longitudinal study. One subject had Down's syndrome and could not be assessed as a result of dementia. The total prevalence of mild to severe hearing loss (33.3% in the 60-70 age group and 70.4% in those over age 70) was comparable to reported data from an ageing population without intellectual disability in the United Kingdom (37%, respectively 60%). However, the proportion of moderate to severe losses might be higher (16.7% vs. 7% in the 60-70 age group and 33.3% vs. 18% in the older age group). Excess impairment was caused by severe congenital and childhood hearing impairment on one hand, and by conductive losses, probably caused by unrecognized chronic middle ear infections, superposed upon presbyacusis, on the other. Impacted ear wax was also a major problem. The incidence of new cases with hearing loss during follow-up was 50%. After individual habituation training hearing aids were used without difficulties by 20 out of 24 subjects. The importance of active screening and treatment of middle ear infections and hearing impairment from a young age onwards, and regular cleaning of the external ear canals is stressed.

Aged↗
PubMed1992

Evaluation of a screening instrument for dementia in ageing mentally retarded persons.

The value of a standardized hetero-anamnestic questionnaire, the Dementia Questionnaire for Mentally Retarded Persons (DMR), as a screening instrument for diagnosis of early dementia, has been evaluated during a longitudinal follow-up of 139 institutionalized ageing mentally retarded subjects. By means of decision analysis, provisional criteria have been derived for a diagnosis, based on absolute DMR scores and on scorechanges through time. For different intellectual levels, separate criteria are required for interpretation of absolute scores, but longitudinal scorechanges might be independent of the intellectual level. The DMR was only evaluated for the mildly and moderately retarded intelligence categories, because of the small number of dementing persons among the severely and profoundly retarded subjects. Causes of false-positivity and false-negativity are considered.

Aged↗
PubMed1992

Hearing loss in middle-age persons with Down syndrome.

Hearing function of 35 institutionalized persons with Down syndrome, age 35 to 62 years, was assessed by means of otoscopy, impedance audiometry, brainstem evoked response audiometry, and pure tone audiometry. Using brainstem evoked response audiometry, we determined response thresholds for 59 ears, which compares favorably with pure tone audiometry (20 ears). We found hearing losses of 20 dB to over 90 dB in 56 of these ears. Hearing loss should be considered and, whenever feasible, excluded as a contributing factor in social and mental deterioration in middle-age persons with Down syndrome.

Acoustic Impedance Tests↗