Lienhard Maeck

Diagnosis of Dementia in Primary Care: A Representative Survey of Family Physicians and Neuropsychiatrists in Germany

Aim: To measure the diagnostic competence of family physicians (FP) and neuropsychiatrists (NP) for moderate dementia. Methods: Written case vignettes describing moderate dementia either of Alzheimer type or vascular type were randomized to a representative sample of 122 FP and 68 NP, corresponding to response rates of 71.8 and 67.3%, respectively. They served as the basis for a structured face-to-face interview. Results: NP and FP did not differ with regard to their diagnostic considerations, however, concerning diagnostic workup. Vascular dementia was much better recognized than dementia of Alzheimer type. Neuropsychological tests and brain imaging would be done by 14.8 and 32.8% of the FP in the case of vascular dementia. In Alzheimer dementia they would apply these methods in 24.6 and 19.7%, respectively. The corresponding numbers for NP were about 60% in both cases for testing and more than 80% for brain imaging. Conclusions: There is still a wide gap between guidelines and practice in primary care. The apparent overdiagnosis of vascular dementia may be one reason for the low drug treatment rates.

Genetic instability in myelodysplastic syndrome: detection of microsatellite instability and loss of heterozygosity in bone marrow samples with karyotype alterations

Using a polymerase chain reaction (PCR)‐based approach, we examined the prevalence of loss of heterozygosity (LOH) and microsatellite instability (MSI) in relation to chromosomal imbalances in myelodysplastic syndrome (MDS). Two of 26 patients displayed MSI (8%), one of them at five loci. LOH was detected in six out of 26 cases (23%), predominantly involving markers IRF1 [5q31] and WT1 [11p]. Two patients displayed a corresponding chromosomal deletion by conventional cytogenetics. Supporting the mutator phenotype hypothesis, a significant coincidence of LOH, MSI and chromosome abnormalities was observed (P < 0·025). Moreover, our data suggest that LOH represents an initial rather than a secondary genetic event in MDS, promoting genetic instability in a subset of patients.

Aβ peptide1–42, Tau protein and S-100B protein level in cerebrospinal fluid of three patients with primary progressive aphasia

Abstract Primary progressive aphasia (PPA) is a clinical syndrome characterized by a slowly progressive aphasia in the absence of accompanying signs of generalized dementia. While non-fluent PPA tends to progress frontally and is usually linked to frontotemporal degeneration, fluent PPA might be associated with both, frontotemporal degeneration or Alzheimers disease. Although recent reports suggest that PPA belongs neuropathologically to the group of tauopathias, cerebrospinal fluid analysis has not been established as a means of diagnosis in PPA so far. In this paper we investigated Aβ peptide1–42 (Aβ1–42), Tau protein and S-100B protein level in the cerebrospinal fluid of three patients with PPA. In all patients Tau protein and S-100B level were slightly elevated, however, Aβ1–42 was found to be in normal range. Thus, our first results point to PPA being neurochemically linked to frontotemporal degeneration.

Dementia diagnostics in primary care: a representative 8-year follow-up study in Lower Saxony, Germany.

Aim: To investigate whether primary-care physicians’ competency regarding dementia diagnostics improved from 1993 to 2001. Methods: In a representative follow-up survey 122 out of 170 (71.8%) family physicians (FPs) were randomly assigned to 2 written case samples presenting patients with slight memory impairment (case 1a: female vs. case 1b: male) and moderate dementia [vascular type (case 2a) vs. Alzheimer’s disease (case 2b)]. Potential diagnostic workup was inquired by a structured face-to-face interview. Results: ‘Basic’ diagnostics like history taking or laboratory investigations were considered in the first place. In case 1, neuropsychological screening was significantly more frequently considered at follow-up (19.3% in 1993 vs. 31.1% in 2001); it still would have been applied rarely in case 2 (2a: 14.1 vs. 14.8%; 2b: 23.5 vs. 24.6%). Neuroimaging remained not to be considered as a standard procedure, and only a minority of FPs would have performed a screening for depression (2001: 1a: 6.7%; 1b: 11.3%; 2a: 0.0%; 2b: 1.6%). Conclusions: With regard to dementia diagnostics in primary care, guideline adherence remained low at follow-up. Structured training efforts aiming at FPs appear to be necessary.

Filial maturity as a predictor for the burden of demented parents' caregivers.

SummaryIn this study, we administered the Louvain Filial Maturity Scale [Marcoen 1993] to 61 adult children of demented elderly. The scores of the seven factors of this scale were compared to the scores of an unselected group of adult children examined by Marcoen. The results were taken into the context with caregiver’s burden, and the effect of filial maturity on parents’ institutionalisation was investigated. Marcoen’s results were confirmed. Only the means of “filial help” and “parental consideration” differed slightly from the means of the unselected group. Overall, filial maturity had no influence on the caregiver’s feeling of burden, but higher “parental consideration” resulted in lower caregiver burden. In addition, adult children with more “filial obligation” continued to care for their parents in the community more often, even when experiencing great burden and stress. However, institutionalisation was caused mainly by parents’ growing needs and increasing behavioural problems. We conclude that “filial maturity” seems to be a very stable concept. Further investigations should focus on the relevance of the Louvain Filial Maturity Scale for caregiving relationship and also on the arrangement of the scale in order to exclude a “pseudo”-stability with regard to burdensome life events and situations.ZusammenfassungIn dieser Untersuchung wurde die Louvain Filial Maturity Scale bei 61 erwachsenen Kindern demenzkranker älterer Menschen eingesetzt. Die Scores der sieben Skalen der Louvain Filial Maturity Scale wichen nicht signifikant von denen der von Marcoen beschriebenen Gruppe ab. Die Ergebnisse wurden in den Kontext der Belastung von Pflegenden gestellt und der Effekt der filialen Reife auf die elterlichen Heimeinweisungen wurde untersucht. Trotz Abweichungen bei den Parametern „filiale Hilfe“ und „elterliche Wertschätzung“ wurden Marcoens Ergebnisse bestätigt. „Filiale Reife“ insgesamt hatte keinen Einfluss auf das Belastungsgefühl von Pflegenden, während eine höhere „elterliche Wertschätzung“ dieses Gefühl reduzierte. Außerdem behielten erwachsene Kinder mit größerem „filialen Verpflichtungsgefühl“ auch trotz eines hohen Belastungsgefühls die ambulante Pflege eher aufrecht. Wir halten „Filiale Reife“ für ein stabiles Konzept, die Louvain Filial Maturity Scale sollte weiter auf ihre Relevanz für Pflegebeziehungen untersucht werden.

Therapie von Verhaltensstörungen bei Menschen mit Demenz

Zusammenfassung: Verhaltensstorungen sind vielgestaltig und haufig bei Demenzen. Ihr Auftreten und Ausmas sind ein Hauptrisikofaktor fur die Heimeinweisung. Mit zunehmender Demenzschwere wird ein Zusammenhang zu Umgebungsfaktoren immer deutlicher. Angehorigeninterventionen beeinflussen auch das Verhalten der Demenzkranken. Die Behandlung erfordert zunachst eine sorgfaltige Analyse auslosender und verstarkender Faktoren. Bestehen Sie fort, so sollte ein Zielsymptom definiert werden und im Behandlungsverlauf dokumentiert werden. Unwirksame Therapien sollten nicht fortgesetzt werden. Pharmakologisch sind Antidementiva als Basistherapie zu prufen. Im Ubrigen haben nicht-anticholinerge Substanzen und atypische Neuroleptika (v. a. Risperidon, Aripiprazol, Olanzapin) eine begrenzte Wirksamkeit. Praparate mit wenig Interaktionen und kurzer Halbwertszeit sind zu bevorzugen. Seitens der nichtpharmakologischen Masnahmen unterscheidet man Ubungs- von sinnesorientierten Verfahren, sowie Validation, Musiktherapie und d...

Differential cellular expression of the human MSH2 protein in normal and myelodysplastic haematopoiesis

Loss of human MSH2 (hMSH2) protein might be involved in the multistep pathogenesis of haematological malignancies associated with genetic instability. Here, we examine cellular hMSH2 expression in bone marrow samples from 10 haematopoietically normal individuals in comparison with nine patients with myelodysplastic syndrome (MDS) [one refractory anaemia (RA), two RA with ringed sideroblasts (RARS), four RA with excess blasts (RAEB) and two RAEB in transformation (RAEB‐T)]. HMSH2 protein was predominantly expressed in myeloblasts and promyelocytes. Blast cells from three patients with RAEB and one with RAEB‐T displayed absent or very low hMSH2 expression. As no correlation between hMSH2 expression and chromosomal aberrations was observed, further genetic events seem to be required to induce karyotype instability.

Why is Competence Assessment Important? Development of the EDCON Consensus Statement

The following chapter serves to underline the importance of developing consensus on the competence of the elderly, especially the demented population. Briefly said, the percentage of the elderly in the population is increasing in modern societies due to the increased life expectancy and reduced birth rates. In consequence an age-associated disease as dementia is going to double its prevalence within the next 20 years. Family and household structures change with more single households, more relationships on distance and shorter lifespan-partnerships. Individual rights and autonomy are increasingly and generally acknowledged as having high priority, maybe also because there seems to be more tolerance to and higher variety of attitudes and beliefs. Last but not least, medical progress has allowed to analyse mental processes with a variety of methods and thus opened opportunities for adequate multi-modal assessments and understanding.