FTLD-TDP with motor neuron disease, visuospatial impairment and a progressive supranuclear palsy-like syndrome: broadening the clinical phenotype of TDP-43 proteinopathies. A report of three cases

<p>Abstract</p> <p>Background</p> <p>Frontotemporal lobar degeneration with ubiquitin and TDP-43 positive neuronal inclusions represents a novel entity (FTLD-TDP) that may be associated with motor neuron disease (FTLD-MND); involvement of extrapyramidal and other systems has also been reported.</p> <p>Case presentation</p> <p>We present three cases with similar clinical symptoms, including Parkinsonism, supranuclear gaze palsy, visuospatial impairment and a behavioral variant of frontotemporal dementia, associated with either clinically possible or definite MND. Neuropathological examination revealed hallmarks of FTLD-TDP with major involvement of subcortical and, in particular, mesencephalic structures. These cases differed in onset and progression of clinical manifestations as well as distribution of histopathological changes in the brain and spinal cord. Two cases were sporadic, whereas the third case had a pathological variation in the progranulin gene 102 delC.</p> <p>Conclusions</p> <p>Association of a "progressive supranuclear palsy-like" syndrome with marked visuospatial impairment, motor neuron disease and early behavioral disturbances may represent a clinically distinct phenotype of FTLD-TDP. Our observations further support the concept that TDP-43 proteinopathies represent a spectrum of disorders, where preferential localization of pathogenetic inclusions and neuronal cell loss defines clinical phenotypes ranging from frontotemporal dementia with or without motor neuron disease, to corticobasal syndrome and to a progressive supranuclear palsy-like syndrome.</p

Alterated diffusion parameters in the brain with kaolin-induced hydrocephalus

Compression of the ECS in the cerebral cortex following induction of hydrocephalus, slows down the diffusion of neuroactive substances in hydrocephalic tissue

Food preference for milk and dairy products

Milk and dairy products constitute an important source of energy and nutrients for humans. Food preferences may significantly influence the actual consumption (and thus nutrition) of people at the population level. The objective of the present large-scale survey was to specify current preferences for milk and dairy products with regard to age and sex. The study was conducted across the Moravia region, Czech Republic, on a sample of 451 individuals divided into 4 age groups: children, adolescents, young adults, and elderly people. A graphic scale questionnaire was administered, with respondents rating their degree of preference for each food item by drawing a mark on a 35 mm line. Out of the 115 items in the questionnaire, 11 items represented dairy products. Data was analysed by means of a general linear model using IBM SPSS Statistics software. Preference for milk was lower in the elderly group than the other groups (P P < 0.01). The overall preference for dairy products (21.6) was lower than the average preference for all foods on the list (22.5). The cross-sectional study revealed intergenerational differences in preferences for specific dairy products, which were most marked in case of cream, processed cheese, blue cheese, and buttermilk. The knowledge of these differences might help promote more focused action at the community level directed at increasing the overall consumption of dairy products in the population

Concomitant antihypertensive medication and outcome of patients with metastatic castration‐resistant prostate cancer receiving enzalutamide or abiraterone acetate

Abstract Background The introduction of novel hormonal therapies represented by enzalutamide (ENZ) and abiraterone acetate (ABI) has reached a great progress in the treatment of metastatic castration‐resistant prostate cancer (mCRPC). The majority of mCRPC patients are elderly suffering from chronic co‐morbidities requiring use of various concomitant medications. In the present study, we focused on impact of concomitant antihypertensive medication on the outcomes of mCRPC patients treated with ENZ or ABI. Methods In total, 300 patients were included and their clinical data were retrospectively analyzed. Results Angiotensin‐converting enzyme inhibitors (ACEIs) represented the only concomitant medication significantly associated with survival. The median radiographic progression‐free survival (rPFS) and overall survival (OS) for patients using ACEIs were 15.5 and 32.3 months compared to 10.7 and 24.0 months for those not using ACEIs (p = 0.0053 and p = 0.0238, respectively). Cox multivariable analysis revealed the use of ACEIs a significant predictive factor for both rPFS (HR = 0.704, p = 0.0364) and OS (HR = 0.592, p = 0.0185). Conclusion The findings of this study suggest an association between the concomitant use of ACEIs and longer survival of mCRPC patients receiving ENZ or ABI therapy