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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

DEMENCIA FRONTOTEMPORAL: LA DEMENCIA MAS PSIQUIATRICA

Posted by Enrique Botia Paniagua - Jefe de Neurología HGMC on July 6, 2015 at 2:15 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.


 

 

 

BIBLIOGRAFÍA

 

  • 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.

 

 

 

 


 

BEATRIZ ESPEJO MARTINEZ
SERVICIO DE NEUROLOGIA HGMC

 


 

Categories: Demencias

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procarbazine is a chemotherapy medication used for the treatment of hodgkin's lymphoma and brain cancers. for hodgkin's it is often used together with chlormethine, vincristine, and prednisone while for brain cancers such as glioblastoma multiforme it is used with lomustine and vincristine. it is typically taken by mouth.

common side effect include low blood cell counts and vomiting. other side effects include tiredness and depression. it is not recommended in people with severe liver or kidney problems. use in pregnancy is known to harm the baby. procarbazine is in the alkylating agents family of medication. how it works is not clearly known.

procarbazine was approved for medical use in the united states in 1969. it is on the world health organization's list of essential medicines, the safest and most effective medicines needed in a health system. in the united kingdom a month of treatment cost the nhs 450 to 750 pounds.

medical uses
when used to treat hodgkin's lymphoma, it is often delivered as part of the beacopp regimen that includes bleomycin, etoposide, adriamycin, cyclophosphamide, vincristine (tradename oncovin), prednisone, and procarbazine. the first combination chemotherapy developed for hodgkin's lymphoma (hl), mopp also included procarbazine (abvd has supplanted mopp as standard first line treatment for hl, with beacopp as an alternative for advanced/unfavorable hl). alternatively, when used to treat certain brain tumors (malignant gliomas), it is often dosed as pcv when combined with lomustine (often called ccnu) and vincristine.

dose should be adjusted for kidney disease or liver disease.

side effects
very common (greater than 10% of people experience them) adverse effects include loss of appetite, nausea and vomiting. other side effects of unknown frequency include reduction in leukocytes, reduction in platelets, reduction in neutrophils, which can lead to increased infections including lung infections; severe allergy-like reactions that can lead to angioedema and skin reactions; lethargy; liver complications including jaundice and abnormal liver function tests; reproductive effects including reduction in sperm count and ovarian failure.

when combined with ethanol, procarbazine may cause a disulfiram-like reaction in some people.

it weakly inhibits mao in the gastrointestinal system, so it can cause hypertensive crises if associated with the ingestion of tyramine-rich foods such as aged cheeses; this appears to be rare.

procarbazine rarely causes chemotherapy-induced peripheral neuropathy, a progressive, enduring, often irreversible tingling numbness, intense pain, and hypersensitivity to cold, beginning in the hands and feet and sometimes involving the arms and legs.
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