[Effectivity of early psychosocial counselling for family caregivers in general practioner based dementia care]. / Hausarztbasierte Demenzversorgung: Effektivität früher psychosozialer Beratung der Angehörigen.

BACKGROUND: Psychoeducation and professional support of family caregivers are regarded as important aspects in dementia care. In the context of a general practitioner's (GP) based dementia care project we investigated if professional counselling after making a diagnosis of dementia can reduce the development of depression in family caregivers. METHODS: 42 patients with dementia and their family caregivers took part in the prospective, randomized study with a follow-up after 18 months. 29 families received psychosocial counselling, the control group was provided an information brochure about dementia including helpful contact addresses. Counselling included education about the illness. Additionally, the current individual needs were identified and assistance was administered if necessary. RESULTS: The counseling was rated as "very helpful" or "helpful" by most of the caregivers as well as by the partaking GPs. While depression rate between counseling and control group did not differ significantly at baseline, the depression rate was significantly increased in the control group after 18 month as compared to the intervention group. CONCLUSION: Despite the small study size we provide first strong evidence that early psychosocial counselling can reduce the development of depression in family caregivers. Early, professional counselling improves the quality of dementia care within a GP-based dementia care setting and it might stabilize and extend the home care duration of dementia patients.

Neuropsychiatric symptoms and brain structural alterations in Fabry disease.

BACKGROUND: Neuropsychiatric symptoms (NPS), mainly cognitive deficits up to dementia and depressive syndromes have been described repeatedly in Fabry disease (FD). However, examinations regarding the pattern, extent, and frequency of the NPS in FD are still lacking. Moreover, the relationship between NPS and brain structural alterations in FD is unknown. The aim of this study was 1) to characterize NPS in a relatively large cohort of adult subjects with FD, and 2) to explore the association of cognitive performance and depressive syndromes with the FD-typical brain structural findings. METHODS: Twenty-five Fabry patients (age 36.5 ± 11.0) with mild to moderate disease involvement and 20 age, gender-, and education-matched healthy controls were extensively studied by neuropsychiatric assessment, structural magnetic resonance imaging, magnetic resonance angiography, and diffusion-tensor imaging. RESULTS: Patients with FD showed deficits only in the attention domain. Clinically relevant depressive syndromes were noted in 60% of the patients. The subgroup of patients with markedly elevated volumes of white matter lesions (not associated with actual stroke; n=7) showed slightly more learning and memory deficits, but no higher depression rate compared to less affected patients. CONCLUSIONS: Against the prevailing assumption, Fabry patients, even those with marked brain structural alterations, showed only mild cognitive deficits. The high frequency of depression in FD is likely to be related to the burden of this chronic multiorganic hereditary disease, but not to the FD-typical brain structural alterations. Longitudinal studies are necessary to clear, if the mild cognitive deficits in FD might precede clinically relevant cognitive decline.

[Improving general practitioners guided dementia care: the pilot project start-modem in Rheinland-Pfalz, Germany]. / Verbesserte ambulante Demenzversorgung. Das hausarztbasierte rheinland-pfälzische Modellprojekt "start-modem".

BACKGROUND: The study examined the ability of trained General Practitioners (GPs) to carry out guideline based diagnostics and therapy of dementia. The article reports the GP-related findings. METHODS: 100 GPs took part in the prospective multi-centre study. 34 GPs started self-guided medical dementia care after attending a special training. The control group of 66 GPs referred patients suspected of dementia to specialist centres. Time of recruiting was 13 months. In case of dementia diagnosis patients and caregivers received professional psychosocial counselling. RESULTS: Trained GPs reported improvements of sensitization and qualification through the project. The cross-linkage of GPs' medical dementia care and psychosocial counselling found a broad acceptance of the participants (GPs, patients, caregivers). 92 of the 156 included patients were diagnosed as dementia. Guideline conformity was violated in 65.4 % of all cases concerning structural imaging and in 30.1 % regarding laboratory tests. "Unspecified Dementia" was the most frequent ICD-10-diagnosis by GPs. CONCLUSION: Despite the broad acceptance of the project a limited guideline-conformity as well as the diagnostic uncertainty suggests that the implemented training is only a first step towards GP guided dementia care. To extend GPs guided dementia care and to improve the quality of GPs dementia diagnostics and therapy sensitization of more GPs for active dementia care as well as supervised practical training will be necessary.

Association between cingulum bundle structure and cognitive performance: an observational study in major depression.

BACKGROUND: Major depression can be regarded as a systemic neurobehavioral disorder resulting from dysfunction of the limbic-cortical networks. The cingulum bundle represents a major association fiber tract of those networks. The aim of our study was to determine the association of brain structural tissue markers of the cingulum bundle and cognitive function in patients with major depression. METHODS: Region-of-interest-based analyses of the middle-anterior and middle-posterior cingulum bundle fractional anisotropy (FA) and mean diffusivity (MD) using color-coded diffusion-tensor imaging and neuropsychological assessment in 14 patients with major depression. RESULTS: FA of the middle-anterior and middle-posterior cingulum bundle was significantly correlated to the performance in a planning and divided attention task. Furthermore, MD of the middle-posterior cingulum bundle was significantly correlated to a planning task. There was no significant correlation between FA and MD of the cingulum bundle and selective attention or memory. CONCLUSIONS: Brain structural tissue markers of the middle-anterior and middle-posterior cingulum bundle were found to be associated with executive functioning and divided attention in patients with major depression. Disconnection within the limbic-cortical networks may underlay cognitive dysfunction in major depression.

Diagnostic utility of different MRI and MR angiography measures in Fabry disease.

BACKGROUND: Neurologic hallmarks of Fabry disease (FD) include small fiber neuropathy as well as cerebral micro- and macroangiopathy with premature stroke. Cranial MRI shows progressive white matter lesions (WML) at an early age, increased signal intensity in the pulvinar, and tortuosity and dilatation of the larger vessels. To unravel the most promising imaging tool for the detection of CNS involvement in FD we compared the diagnostic utility of the different MR imaging findings. METHODS: Twenty-five clinically affected patients with FD (age 36.5 +/- 11.0) and 20 age-matched controls were investigated by structural MRI, MR angiography, and diffusion tensor imaging (DTI). Individual WML volumes, global mean diffusivity (MD), and mean cerebral artery diameters were determined. RESULTS: Using receiver operating characteristic analyses, enlarged diameters of the following cerebral arteries significantly separated patients with FD from controls: middle cerebral artery: area under curve (AUC) = 0.75, p = 0.005; posterior cerebral artery: AUC = 0.69, p = 0.041; carotid artery: 0.69, p = 0.041; basilar artery: AUC = 0.96, p < 0.0005. A total of 87% of the individuals were correctly classified by basilar artery diameters (sensitivity 95%, specificity 83%). WML volumes and global MD values did not significantly separate patients from controls. CONCLUSIONS: With an accuracy of 87%, basilar artery diameters were superior to all other MR measures for separating patients with Fabry disease (FD) from controls. Future studies should adopt basilar artery measurements for early detection and monitoring of brain involvement in FD. Moreover, further investigations should reveal if the dilated vasculopathy in FD could be a screening marker to detect FD in a cohort of other cerebrovascular diseases, especially in cryptogenic stroke.

Experimental evidence for a motivational origin of cognitive impairment in major depression.

BACKGROUND: Diagnostic criteria and empirical evidence support the existence of cognitive deficits in depression. However, depressed mood, loss of interest and low self-efficacy might influence cognitive performance. METHOD: Goal-setting instructions were used to promote motivation in depressed patients and control subjects during neuropsychological assessment. The resulting performance was compared with performance using standard instructions. Sixty in-patients with non-psychotic unipolar depression and 60 age- and education-matched healthy control subjects were assessed with standard neuropsychological tests [the Auditory Verbal Learning Test (AVLT), the Digit Symbol Test (DST), the Regensburg Word Fluency Test (RWT), and the Number Combination Test (Zahlen-Verbindungs-Test, ZVT)] using either goal-setting or standard test instructions. RESULTS: Depressed patients showed lower baseline performance and lower generalized self-efficacy (p<0.0005) than controls. However, goal-setting instructions significantly improved patients' memory performance by 10% [AVLT: F(5, 54)=3.611, p=0.007] and psychomotor performance by 13% [ZVT: F(3, 56)=3.667, p=0.017]. Consequently, patients and control subjects demonstrated similar results when goal-setting instructions were applied. Goal-setting instructions showed a statistical trend, increasing patients' performance in the DST by 12% [F(1, 58)=2.990, p=0.089], although their verbal fluency measured by the RWT did not increase. No significant correlations of increased performance with generalized self-efficacy were found. CONCLUSIONS: Cognitive deficits in depressed patients are influenced by motivational shortcomings. Because generalized self-efficacy failed to correlate to increased test performance, future research needs to disentangle the effective components of goal-setting instructions. Task-specific self-efficacy as well as enhancement of task-focused attention might underlie the significant goal-setting effect in depressed patients.

Voxel based analyses of diffusion tensor imaging in Fabry disease.

BACKGROUND: Fabry disease (FD) is a lysosomal storage disorder associated with marked cerebrovascular disease. Conventional MRI shows an extensive load of white matter lesions (WMLs) which may already be present at an early stage in the disease. OBJECTIVE: Investigator independent and sensitive quantification of structural changes in the brain in clinically affected men and women with FD. METHODS: We performed a voxel based analysis of diffusion tensor images (DTI) in 25 patients with FD and 20 age matched normal controls. RESULTS: DTI revealed significant increases in cerebral white matter mean diffusivity (MD) in patients with FD, which were pronounced in the periventricular white matter. Even the subgroup of patients without significant WMLs load (n = 18) showed increased diffusivity in the cerebral white matter. In gray matter areas, MD elevation was detected only in the posterior part of the thalamus, independent of the visible pulvinar alterations on T1 weighted images. Voxel based fractional anisotropy measurements did not differ significantly between patients and controls. CONCLUSIONS: The present study demonstrates the clinical feasibility of voxel based analysis of DTI as a sensitive tool to quantify brain tissue alterations in FD. The pattern of increased brain tissue diffusivity is probably due to microangiopathic alterations, mainly affecting the long perforating arteries.