Publication: Potencial terapéutico del sistema endocannabinoide en el espectro ELA-DFT
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2021-11-26
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Universidad Complutense de Madrid
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Abstract
La demencia frontotemporal (DFT) es un término clínico que abarca un grupo heterogéneo de trastornos progresivos y tempranos caracterizados por la degeneración de los lóbulos frontal y temporal, lo que se traduce en trastornos emocionales y comportamentales, alteraciones en el lenguaje y déficit cognitivo. Durante las últimas décadas la DFT ha cobrado una gran relevancia, en parte, por sus características solapantes con otra enfermedad neurodegenerativa: la esclerosis lateral amiotrófica (ELA). La ELA es una enfermedad neurodegenerativa crónica y progresiva que cursa con la degeneración y muerte de las motoneuronas superiores e inferiores, lo que conduce a una atrofia y parálisis progresiva de la musculatura esquelética. En la actualidad ambas patologías no se conciben como entes individuales, sino como extremos de un espectro clínico continuo caracterizado por la presencia de alteraciones genéticas y patológicas comunes, entre las que cabe destacar la agregación proteica, la neuroinflamación o la disrupción de los mecanismos de autofagia. Uno de los hallazgos que llevó a establecer esta relación fue la identificación en 2006 de la proteína TDP-43 como el principal componente de las inclusiones citoplasmáticas presentes tanto en neuronas de pacientes de ELA (97% de los casos) como de DFT (45% de los casos). A pesar de los numerosos avances en el conocimiento de ambas patologías, no existe ninguna terapia eficaz para prevenir, retrasar o frenar la degeneración neuronal. Por este motivo surge la necesidad urgente de buscar nuevas estrategias farmacológicas que consigan enlentecer, o en el mejor de los casos, frenar la progresión de la enfermedad en este grupo de pacientes cuya esperanza de vida no supera, en muchos casos, los 3 años desde el inicio de la sintomatología...
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...
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...
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Tesis inédita de la Universidad Complutense de Madrid, Facultad de Medicina, leída el 15-07-2021