Epidemiological data on Werdnig-Hoffmann disease in Germany (West-Thüringen)

This study contains the largest body of epidemiological data on Werdnig-Hoffmann disease (acute infantile spinal muscular atrophy; ASMA) in West-Thüringen in Germany. The incidence of ASMA was calculated to be 1 in 10202 live births. The prevalence was 1 in 595362 of the general population (as of 31 December 1987). The study gives an unexpectedly high incidence rate confirming the suggestion that ASMA in Central and Eastern Europe might be more frequent than in Western Europe. However, we consider that this high incidence rate in West-Thüringen is a result of the almost complete ascertainment made possible because of the well-organised and centralised health system existing in Thüringen over the last few decades.     

Generic quality of life assessment in dementia patients: a prospective cohort study

Background  

Quality of life (QoL) is increasingly used to characterize the impact of disease and the efficacy of interventions.     

Sensitivity of different stimulus-timing strategies for the detection of small excitations in noisy spike train data

There are several different strategies to control the timing of a stimulus with respect to the ongoing discharge during the recording of neuronal stimulus-response characteristics. One possible strategy consists of delivering stimuli in such a way that a constant pre-stimulus spike density is reached. Another strategy enforces spike application with a constant stimulus latency after a spontaneous discharge. In this paper the sensitivity of these different strategies for statistical verification of small excitatory response components was investigated. It was found that the difference between observed poststimulus spike distribution and expected spike distribution under the null hypothesis of no stimulus effect was larger using a constant-stimulus-latency (CSL) strategy with an appropriate value for the stimulus latency. Thus, the statistical verification of neuronal response components is clearly facilitated if a CSL strategy is used. This superiority of the CSL strategy is marked, especially for small excitations at neurons discharging slowly with low discharge variability.