The cause of motor neuron death in amyotrophic lateral sclerosis (ALS) remains a mystery. Initial implications of neurotrophic factor impairment involved in disease progression causing selective motor neuron death were brought forward in the late 1980s. These implications were based on several in vitro studies of motor neuron cultures in which a near to complete rescue of axotomized neonatal motor neurons in the presence of supplementary neurotrophic factors were revealed. These findings pawed the way for extensive investigations in experimental animal models of ALS. Neurotrophic factor administration in rodent ALS models demonstrated a remarkable effect on survival of degenerating motor neurons and rescue of axotomized motor neurons, both in vivo and in vitro. In the absence of efficient therapy for ALS, some of these promising neurotrophic factors have been administered to groups of ALS patients, as they appeared available for clinical trials. Up to date, none of tested factors has lived up to expectations, altering the outcome of the disease. This review summarizes current findings on neurotrophic factor expression in ALS tissue and these factors’ potential/debatable clinical relevance to ALS and the treatment of ALS. It also discusses possible interventions improving clinical trial design to obtain efficacy of neurotrophic factor treatment in patients suffering from ALS.

A divergence in earlier multiple sclerosis (MS) mortality rates observed within Europe, prompted us to determine the MS mortality rate in Austria and several European countries. Our aim was to examine the temporal and geographical variations within Austria and to determine future MS mortality rates based on a projection model. MS mortality data set, differentiated by age groups, sex, and region at death for the period 1970-2001 were obtained. Prognostic MS mortality trends for the period 2002-2020 were estimated using the simultaneous multiple cause-delay (SIMCAD) method. Our findings indicate a decline (47%) in the MS mortality rate from 1.41 (1970-79) to 0.96 (1980-89) and 0.70 (1990-2001) per 100 000 in Austria during the 32-year period observed. Conversely, the scenarios of our projection for the period 2002-2020, reveal an increasing MS mortality rate. The median age at death because of MS increased with 0.7 years for men and 2.9 years for women during the observed period (1970-2001). Austria, like many other European countries, has experienced a decreasing MS mortality rate over the last three decades. An increased MS mortality rate is however expected over the next decades in Austria. This increase will be most prominent in the elderly population cohorts because of demographic shifts.

Recent increasing incidence and prevalence rates of Huntington's disease (HD), a fatal neurodegenerative disorder, prompted us to investigate the epidemiological dynamic of HD in Austria during the period 1970--2001. Our study demonstrated a stable HD mortality rate throughout Austria of 0.125 per 100,000 individuals during the investigated period. The median age at death from HD was 56.5 years for both sexes and remained stable during the entire period observed, indicating no prolonged survival. An above average mortality rate from HD was observed in the north-eastern parts of Austria. Our data reflect the epidemiology of HD prior to and after the availability of genetic testing and provide a solid baseline for future investigations on the epidemiology of HD.