Potencial terapéutico del sistema endocannabinoide en el espectro ELA-DFT

Zusammenfassung

Frontotemporal dementia (FTD) is a clinical term that encompasses a heterogeneous group of early, progressive disorders characterised by degeneration of the frontal and temporal lobes, resulting in emotional and behavioural disturbances, language impairment and cognitive deficits. During the last decades, FTD has gained relevance, partly due to its overlapping characteristics with another neurodegenerative disease: amyotrophic lateral sclerosis (ALS). ALS is a chronic, progressive neurodegenerative disease that causes degeneration and death of upper and lower motor neurons, leading to progressive atrophy and paralysis of the musculature. Today, both pathologies are not only conceived as individual entities, but also as extremes of a continuous clinical spectrum characterised by the presence of common genetic and pathological alterations, including protein aggregation, neuroinflammation and disruption of autophagy mechanisms. One of the findings that led to this association was the identification in 2006 of the TDP-43 protein as the main component of the cytoplasmic inclusions present in neurons of both ALS (97% of cases) and FTD (45% of cases) patients. Despite numerous advances in the knowledge of both pathologies, there is no effective therapy to prevent, delay or stop neuronal degeneration. For this reason, there is an urgent need to search for new pharmacological strategies to slow down or, in the best of cases, stop the progression of the disease in this group of patients whose life expectancy in many cases does not exceed 3 years from the onset of symptoms...