RESUMEN
La demencia por cuerpos de Lewy (DCL) es una enfermedad neurodegenerativa, que supone en torno al 10-25% de todas las demencias de la población general y es la segunda causa de demencia degenerativa, en el anciano, tras la enfermedad de Alzheimer. Se caracteriza clínicamente por deterioro cognitivo, con rasgos de demencia frontal, acompañado de: fluctuaciones, parkinsonismo y alucinaciones visuales. Su confirmación diagnóstica se realiza por anatomía patológica posmortem: presencia de abundantes cuerpos de Lewy (CL) en las neuronas de la corteza y en otras zonas cerebrales. Los CL son inclusiones intracitoplasmáticas eosinófilas, esféricas y están constituidos por más de 20 componentes proteicos. Se han definido, en el año 2005, unos criterios diagnósticos para facilitar su reconocimiento y poder etiquetar a los pacientes de posible o probable DCL. La causa de la DCL es desconocida; se supone que, al igual que en la enfermedad de Parkinson, influyen factores ambientales y genéticos. No hay ninguna prueba complementaria ni marcador biológico específico de esta enfermedad, pero la combinación de biomarcadores, los tests neuropsicológicos y las pruebas de neuroimagen son de especial ayuda en el diagnóstico. El tratamiento de la enfermedad es complejo; porque los fármacos que mejoran unos síntomas pueden empeorar otros. En cualquier caso solo hay posibilidad de tratamiento sintomático (AU)
Dementia with Lewy bodies (DLB) is a neurodegenerative disease, accounting for about 10-25% of all dementias in the general population and is the second most common type of degenerative dementia in elderly people after Alzheimer's disease. Clinically, it is characterized by cognitive impairment (with features of frontal dementia) accompanied by fluctuating cognition, parkinsonism and visual hallucinations. Diagnostic confirmation is made by pathologic autopsy: abundant presence of Lewy bodies (LB) in the neurons of the cortex and other areas in the brain. The LB are eosinophilic intracytoplasmic inclusions, spherical and consist of more than 20 protein components. Diagnostic criteria to facilitate recognition of patients with possible or probable DLB were defined in 2005. The etiology of DLB is unknown, although is assumed that, environmental factors and genetic influence, like in Parkinson's disease. There are not additional evidence or specific biological markers of this disease, but neuropsychological and neuroimaging tests are helpful in diagnosis. The treatment of the disease is difficult, there is no cure, only symptomatic. Some drugs may improve some symptoms but also can worse others (AU)
Asunto(s)
Humanos , Masculino , Femenino , Enfermedad por Cuerpos de Lewy/genética , Enfermedad de Alzheimer/genética , Trastornos del Conocimiento/psicología , Trastornos de la Visión/diagnóstico , Neuronas/citología , Tronco Encefálico/anomalías , Análisis del Polimorfismo de Longitud de Fragmentos Amplificados/métodos , Enfermedad por Cuerpos de Lewy/patología , Enfermedad de Alzheimer/patología , Trastornos del Conocimiento/genética , Trastornos de la Visión/complicaciones , Neuronas/clasificación , Tronco Encefálico/fisiopatología , Análisis del Polimorfismo de Longitud de Fragmentos Amplificados/normasRESUMEN
INTRODUCTION: In Parkinson's disease there are patients with isolated and multiple cognitive impairment, and their cognitive performance ranges from normal to an advanced degree of dementia. Most patients present an executive deficit, either in isolation or combined with other cognitive disorders, which is considered to be the most characteristic aspect of the disease, and 30-40% of those affected will end up with a clinically-defined dementia. DEVELOPMENT: The presence of a mild cognitive disorder in patients with Parkinson means that the risk of dementia appearing at some time during the development of the disease is high. The dementia associated with Parkinson's disease is specifically related with neuropsychiatric signs and symptoms, which may have three possible explanations: disorders affecting the mesolimbic pathways, diffuse limbic and cortical compromise, or associated Alzheimer-type phenomenology. Psychotic episodes tend to present more often in patients with dopaminergic treatment and the clinical spectrum of Parkinson-related psychosis covers visual illusions, visual-audio-olfactory hallucinations, delirium and severe paranoid hallucinatory psychosis. All the antiparkinsonian drugs can give rise to hallucinations and psychosis, but the dopamine agonists are the ones with the greatest capacity to do so. CONCLUSIONS: In managing these problems, it is crucial for prevention as well as diagnosis and treatment to be carried out as soon as they are detected. Doses of antiparkinsonian drugs must be reduced, although this is not usually enough, and so it will be necessary to associate atypical antipsychotics, which act mainly on 5-HT receptors and, in most cases, do not produce D2 blockage.
