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Neurología Mancha-Centro

Servicio de Neurología del Area Mancha Centro - Hosp. General Mancha Centro y Hosp. General de Tomelloso

Blog de Neurologia Post New Entry


Posted by Beatriz Espejo Martinez on July 6, 2015 at 3:50 PM


La demencia frontotemporal, en términos de prevalencia, es la segunda demencia degenerativa en menores de 65 años. En mayores de 65 años se sitúa en tercera posición. Es probable que esta estimación se encuentre infravalorada debido a que los síntomas que la caracterizan son frecuentemente difíciles de identificar.


Clínicamente, esta demencia se define como un conjunto de síndromes caracterizados por una alteración progresiva del comportamiento y/o lenguaje, en relación con una atrofia focal de los lóbulos frontal y temporal. En este contexto, existen tres variantes fundamentales. La más frecuente de ellas es la variante conductual, donde el trastorno de comportamiento predomina sobre la afectación de otras esferas.


Es definida como un cambio progresivo en la personalidad y el comportamiento (3 primeros años), donde la memoria y capacidades visuoespaciales están relativamente preservadas en estadios iniciales (ver tabla 1, criterios de consenso 2007 Rascovsky). El paciente carece de introspección, es decir, no es consciente de lo que le está pasando, y las exploraciones física y neurológica pueden ser inicialmente normales, por lo que se requiere un estudio más amplio, detallado y dirigido a su búsqueda, en el que es fundamental la entrevista con los familiares.


Durante años se ha prestado gran atención al deterioro cognitivo asociado a la Enfermedad de Alzheimer (alteración de la memoria como síntoma primordial asociada en su evolución a afectación de otras áreas cognitivas como la orientación o el lenguaje), dejando relegados otros déficits dentro de una demencia que pueden causar una grave pérdida de funcionalidad como son las alteraciones conductuales. Además, la presencia de éstas sin afectación cognitiva asociada ha conllevado una importante confusión y frecuentemente a clasificarlas dentro de un amplio rango de patologías psiquiátricas (ej: trastornos afectivos, etc.).


A día de hoy, el tratamiento de esta enfermedad neurodegenerativa sigue siendo sintomático, es decir, dirigido a paliar la clínica comportamental que afecta primariamente al paciente y secundariamente a sus cuidadores principales, haciendo que la convivencia pueda ser, en muchos casos, insostenible. Sin embargo, la investigación no se detiene y esperemos que en un futuro próximo nos aporte nuevos tratamientos dirigidos a su prevención y/o resolución.


El pronóstico vital de la Demencia Frontotemporal varía entre los 6-11 años desde el inicio de la clínica y 3-4 años tras su diagnóstico. Los enfermos tienen una menor supervivencia y mayor rapidez de deterioro cognitivo y funcional que el observado en la Enfermedad de Alzheimer.


  • Behavioral form of frontotemporal dementia (DFTc). A literature review and recommendations for early diagnosis. Acta Neurol Colomb 2010;26:Sup (3:1):29-38). Rascovky,
  • RASCOVSKY K, SALMON DP, LIPTON AM, LEVERENZ JB, DECARLI C, JAGUST WJ, ET AL. Rate of progression differs in Frontotemporal Dementia and Alzheimer’s Disease. Neurology. 2005; 65: 397- 403.
  • RASCOVSKY K, HODGES JR, KIPPS CM, JOHNSON JK, SEELEY WW, MENDEZ MF, ET AL. Diagnostic criteria for the behavioral variant of frontotemporal dementia (bvFTD): current limita¬tions and future directions. Alzheimer Dis Assoc Disord. 2007; 21: S14-S18.



Categories: Demencias

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