[Darier and Ferrand dermatofibrosarcoma. About 7 cases at the Ouagadougou teaching hospital, Burkina Faso]. / Le dermatofibrosarcome de Darier et Ferrand (DFDF): A propos de 7 cas au CHU de Ouagadougou, Burkina Faso.

The authors report 7 cases of dermatofibrosarcoma of Darier and Ferrand over a 7 year- period at the Yalgado-Ouédraogo teaching hospital, that is to say an average of 1 case per year. A male predominance was noted (4 males and 3 females), average age: 48; 5 recurrences out of 7, with an average delay of 3 years for recurrence, preferentially localised on the trunk (5 cases) and thigh (2 cases). They emphasize the importance of wide surgical exeresis and histopathology essential to confirm the diagnosis. Finally they point out the importance of clinical surveillance because of frequent recurrences.

Evaluation of the risk factors of asymptomatic vertebral fractures in postmenopausal women with osteopenia at the femoral neck.

OBJECTIVES: To identify risk factors of asymptomatic vertebral fracture (aVF) in postmenopausal women with osteopenia at the femoral neck and to evaluate the association between the number of aVFs and the risk of major and hip osteoporotic fracture calculated with the FRAX(®) algorithm. STUDY DESIGN: Epidemiological case-series study with data collected transversally. RESULTS: 728 postmenopausal women with osteopenia were included: 284 (39.0%) had aVF, of whom 200 (70.4%) had prior fragility fractures (FF). The likelihood of having an osteoporotic fracture in the next 10 years increased significantly with the number of aVF. The percentage of women with height loss, which was assessed as the difference between the greatest height reported by participants and that measured at inclusion, was higher in women with an aVF (OR 3.77, 95% CI 2.75-5.16, p<0.05). Multivariate analysis showed that prior FF, height loss and race were factors associated with the presence of aVF. CONCLUSIONS: In this group of postmenopausal women with osteopenia at the femoral neck, the presence of an aVF correlated with a higher risk of estimated major osteoporotic and hip fractures as calculated using the FRAX(®) algorithm. Height loss and prior FF were associated with the presence of aVF.

A combination of tests for the diagnosis of dementia had a significant diagnostic value.

OBJECTIVE: To study the diagnostic parameters of a number of instruments for a diagnosis of dementia in general practice and the added diagnostic value of these tests. STUDY DESIGN AND SETTING: Cross-sectional diagnostic research in general practice. PARTICIPANTS: 152 persons aged 65 plus. The Mini-Mental State Examination (MMSE), the Clock Drawing Test, the ADMP scale, the Timed Up and Go Test, the Extrapyramidal Sign Scale, the Behavior Observation Scale, the Poon-Baro-Wens computer battery, and the Cognitive Drug Research Computerized Assessment System were evaluated against the Dutch version of the Cambridge Examination for Mental Disorders of the Elderly (CAMDEX-N). Diagnostic characteristics were calculated with their 95% CI. Using forward stepwise logistic regression analysis, a model was built with CAMDEX-N as the dependent variable and the tests under study as independent variables. Area under the curve was the main parameter for the comparisons. RESULTS: The main diagnostic gain results from age and ADMP, followed by the Clock Drawing Test. Subsequent addition of the MMSE and computer tests results in modest additional gain only. The final model including five tests has an area under the curve of 0.95. CONCLUSION: Sophisticated neuropsychological computerized tests have little added value in the diagnostic work-up of dementia in general practice. Basic clinical tests used in an appropriate sequence can be very valuable in establishing the diagnosis of dementia.

Prophylactic approach for asymptomatic post-menopausal women: osteoporosis.

To consider what a correct preventive approach for osteoporosis should be in the management of the asymptomatic post-menopausal woman. Literature review and opinions on this issue shared by a group of professionals with wide clinical experience in health care for peri- and post-menopausal women. There is agreement that osteoporosis combines most of the ideal requirements for the application of a preventive strategy. The natural evolution of this pathology leads to serious events, fractures, but it provides various opportunities for prevention. Osteoporosis can be avoided and in particular, fractures due to bone fragility and their consequences should be avoided. Nowadays, there are different courses of action (pharmacological or otherwise) with proven effectiveness for the prevention osteoporosis and osteoporotic fractures. However, long-term action is required for a broad segment of the population, so a certain strategy is necessary to guide clinical decisions for different profiles of women. There is little data in the literature to justify a different preventive approach, depending on the presence or absence of vasomotor symptoms, but clinical experience shows that their absence is not associated with a lower osteoporosis risk. Different strategies have been tried for the prevention of osteoporosis and its complications and some of them might be effective, but there is no analysis with conclusive results. A preventive strategy for osteoporosis should be included in the management of asymptomatic post-menopausal women, because this is an avoidable pathology and the absence of vasomotor symptoms does not reduce the risk of its development. Nevertheless, a well-designed cost-benefit analysis is needed to justify the implementation of any strategy at a community level, because adverse effects and economic cost could exceed the benefits obtained in low fracture risk populations.

Correlations between triplet repeat expansion and clinical features in Huntington's disease.

OBJECTIVE: To investigate possible correlations between the length of the (CAG)n trinucleotide repeat in Hungtington's disease gene IT15 and clinical features (age at onset, symptoms at onset, and mode of progression) in Huntington's disease. DESIGN: In 59 patients with Huntington's disease, the expansion of the (CAG)n trinucleotide repeat was determined and clinical data were obtained retrospectively. SETTING: The Center for Human Genetics, affiliated with a university hospital. PATIENTS: All patients belonged to an initial group of 248 individuals tested in an indirect predictive testing procedure. RESULTS: A good correlation was found between the expansion of the (CAG)n trinucleotide repeat and the age at onset (r = -.71). No correlation was found between the repeat length of the normal allele and the age at onset. No correlations were found between repeat expansion and other clinical features, such as the nature of the symptoms at onset (neurologic, psychiatric/cognitive, or both) and the mode of progression. CONCLUSION: Factors that determine the nature of symptoms at onset and the mode of progression of Huntington's disease seem to be operating independently of the (CAG)n trinucleotide repeat in gene IT15.

Neuropathology of Huntington's chorea. Studies of the ventrobasal complex of the thalamus.

In seven cases of Huntington's chorea, the ventrolateral thalamus was studied by quantitative cytometry. A selective 50 percent atrophy of microneurons (internuncial cells) was found while the macroneurons did not show significant atrophy. Thalamic microneurons might be presynaptic and postsynaptic inhibitory cells. Their specific atrophy in Huntington's chorea thus could be related to the known decrease of gamma aminobutyric acid (GABA) in Huntington's chorea.

Tiapride versus placebo: a double-blind comparative study in the management of Huntington's chorea.

A double-blind, placebo-controlled, crossover trial was carried out in 29 patients with Huntington's chorea to evaluate the effectiveness and tolerance of high doses of tiapride in the management of neurological symptoms. Patients were allocated at random into 3 groups to receive 3 g tiapride daily for two periods of 3 weeks either preceded, interrupted or followed by a 3-week period on placebo. Patients were assessed on entry and at the end of each treatment period using a battery of tests designed to measure choreatic movements, motor skills, recognition and reaction times, and mental state. Twenty-three patients completed the trial; only 2 of the 6 drop-outs withdrew because of drug-related side-effects (sedation and extrapyramidal signs). The results showed that tiapride treatment significantly improved choreatic movements and motor skills and these findings were supported by the objective psychometric measurements. Overall evaluation of effectiveness by the physician and patients indicated the significant superiority of tiapride over placebo. Treatment was generally well tolerated and although there were significantly more reports of sedation and extrapyramidal signs whilst patients were receiving tiapride, these effects were rated as mild in most cases and did not interfere with treatment.

Sense of coherence, coping and caregiver role overload.

The focus of the present study is to examine the relationship between Antonovsky's Sense of Coherence (SOC), the nature of patient pathology, situational coping responses, and role overload in Belgian primary caregivers to dementing and nondementing chronically ill family members (n = 126). The hypotheses that caregivers with a strong SOC are likely to cope in situationally-appropriate ways were confirmed. Sense of Coherence appears to have a protective effect, in the sense of being related to management of the meaning of the situation, the selection of realistic coping strategies, and the avoidance of potentially maladaptive or unhealthy behaviors. Multi-variate analyses revealed that SOC alone predicted 29% of the variance in role overload for caregivers to dementing patients, while strategies to manage the situation and symptoms of distress were the only significant, yet maladaptive, coping responses for caregivers to nondementing patients. It is arguable that these findings indicate a threshold effect of the nature of patient disability on the protective effects of the sense of coherence. The multi-dimensional impact of caring for a patient with a dementing disorder exerts a unique and particular strain, one that requires caregivers to be able to cope by redefining the meaning of their relationship with their dependent. The theoretical rationale for the hypothesized threshold effect is explored.

[Aspects of cognitive functioning in the aged. A transversal study]. / Aspecten van het cognitief functioneren van bejaarden. Een transversaal leeftijdsonderzoek.

In the context of a WHO study on psychosocial needs of the elderly, a sample of 519 subjects from the region of Louvain was psychometrically tested. This sample consisted of men and woman between 55 and 94 years of age, most of them living at home. The test battery was composed of ten verbal and nonverbal tests and tasks currently used in psychogeriatric clinical practice but lacking validation and normative data for those aged populations. This study (psychometric testing took place in 1979-1980) was intended as a first step in a follow up program to gather more definite information about mental deterioration. Up to now it can be considered however as a cross-sectional study, providing normative data for the assessment of cognitive functioning in aged individuals up to 94 years. The decline in performances, found on all these tests, reflect generation as well as aging effects; the increasing proportions of aged subjects failing on the tests however, seem to indicate a high occurrence of partial or global cognitive deficits in elderly people beyond the eighth decade.

The diagnosis and classification of depressions.

The International Calassification of Diseases and other contemporary classifications of depression are discussed, together with some general and practical classification problems. Unambiguous operational criteria need to be used for establishing diagnostic validity and comparability. There is widespread agreement on the distinction between Type A and Type B depressions and between unipolar and bipolar illness.

The interaction between neuroleptic treatment and sociotherapeutic approach in chronic schizophrenics. Investigations with haloperidol, penfluridol and pimozide.

In the modern treatment of chronic schizophrenic patients two major trends can be outlined: sociotherapy and psychopharmacotherapy. Therefore it is an important question whether these two therapeutic approaches interact in a positive or a negative way. Selected examples of experiments about the interaction of haloperidol, penfluridol and pimozide with sociotherapy are given and discussed. It is also demonstrated that the learning capacity of the patients depends on the particular type of neuroleptic.

The prevalence of dementia in Belgium: a population-based door-to-door survey in a rural community.

The project Epidemiology Research on Dementia in Antwerp (ERDA) estimated the prevalence of dementia in a random, population-based sample, stratified for age and sex. The sample of 1,736 elderly was screened at home with the Mini-Mental State Examination. All elderly under the cutoff of 23-24/30 got a diagnostic examination with the Cambridge Mental Disorders of the Elderly Examination and the DSM-IIIR criteria. The prevalence of dementia in the population above 65 years was estimated at 9%. The following age-specific prevalences of dementia (included mild dementia) were found in the age-groups 65-69, 70-74, 75-79, 80-84, 85+: 0.6, 5.1, 7.6, 16.2 and 33.6%. The prevalence of at least moderate dementia was 0.3, 3.9, 4.0, 11.2 and 25.0%, respectively. The prevalence of dementia, vascular dementia and dementia of the Alzheimer type was markedly higher in women than in men.

Double-blind trial of suloctidil versus placebo in moderate to severe mental deterioration.

A double-blind, parallel group, placebo-controlled study was carried out in 30 elderly patients with moderate to severe mental deterioration to assess the effect of suloctidil on their mental condition. A battery of clinical and psychometric evaluations failed to demonstrate any significant differences between the two groups at the end of a 6-month treatment period during which patients received either 200 mg suloctidil or placebo 3-times daily. However, further analysis of the results showed that in the sub-group of patients with moderate mental deterioration on entry, suloctidil treatment produced significant improvement from baseline in the Rey 15 words test and the results were significantly different from those in patients with severe mental deterioration. No consistent changes were observed in the placebo group.

Epidemiology research on dementia in Antwerp, Belgium.

Epidemiological research on dementia in Belgium started in 1990 with a prevalence study. In the first phase of the MMSE was used for screening a random sample, stratified by age, of 1,800 aged people. In the second phase the diagnostic work was done by a psychiatrist using the CAMDEX. An incidence study will start after 2 years. Potential risk factors will be examined in a case-control study.

Heparan sulfate expression patterns in the amyloid deposits of patients with Alzheimer's and Lewy body type dementia.

Heparan sulfate (HS), along with serum amyloid P component, has been identified in all types of amyloid investigated so far, regardless of the type of amyloid protein deposited. To assess whether unique or specific HS proteoglycans (HSPGs) may be involved in the formation of these lesions, we have investigated the accumulation of several distinct HSPG epitopes in the cerebra of patients with different forms of neurodegenerative disease. A panel composed of several antibodies revealed distinctive patterns of HSPG accumulation. In patients with dementia of the Lewy body type, the burned-out-type plaques and preamyloid-type plaques were strongly stained by both the anti-HS 'chain' and anti-HS 'stub' antibodies, but by none of the available anti-core protein antibodies. In Alzheimer's disease, the preamyloid-type plaques, dense-cored-type plaques, neuritic-type plaques and the neurofibrillary tangles were stained by the anti-'stub' antibody. The anti-'chain' and the anti-core protein antibodies, in contrast, failed to stain the preamyloid-type plaques and burned-out-type plaques, but stained the neuritic-type plaques in these patients. These data suggest differences in the types of HS and HSPG (fragments) that accumulate in amyloid lesions that may hallmark neurodegenerative disorders of different etiologies.

How do general practitioners diagnose dementia?

By means of a qualitative study, we set out to generate hypotheses about the way general practitioners (GPs) diagnosed dementia. We looked for triggers for the diagnosis of dementia. Ten GPs were interviewed about four dementia cases taken from their own practices. GPs are more concerned with treatment than with the diagnosis of dementia as such, as diagnosis is performed in relation to treatment. The most important aid was the evolution of the clinical tables based on the information of caregivers. Important triggers were changes of activities of daily life function, behaviour and cognition. Acute illness and loss of the key caregiver were relevatory moments. Recommendations are made for the improvement of the diagnostic abilities of GPs.

Maintenance treatment of chronic schizophrenic patients. A study with the long-acting thioxanthene derivative, cis(Z)-clopenthixol decanoate-sordinol depot.

The clinical effect of clopenthixol decanoate has been assessed in a 5-month controlled including 21 hospitalized chronic schizophrenic patients. The ratings were done with BPRS, NOSIE 30, the two psychological tests of WAIS and Grübaum, and the rating scale of Simpson & Angus to assess extrapyramidal side effects. Clopenthixol decanoate was found an effective and long-acting antipsychotic compound with few autonomic and neurological side effects. Compared with previous maintenance treatment it also showed a positive influence on depression and facilitation of the social adaptation of the patients.