Identificación clínica y patológica de las diversas formas de la enfermedad de Creutzfeldt Jakob en Chile

Background: The identification of clinical and pathological forms of Creutzfeldt Jakob Disease (CJD) started with the first cases of the disease. Genetic and biomolecular prion status assessment are allowing now a better classification. Aim: To identify the clinical forms of the disease that exist i...

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Autores principales:	Cartier R,Luis, Quiroz Z,Gonzalo, Leiva H,Marcelo, Vergara R,Carmen
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2012
Materias:	Ataxia Creutzfeldt Jakob disease Pathology, clinical
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872012000200003
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spelling	oai:scielo:S0034-988720120002000032013-04-11Identificación clínica y patológica de las diversas formas de la enfermedad de Creutzfeldt Jakob en ChileCartier R,LuisQuiroz Z,GonzaloLeiva H,MarceloVergara R,Carmen Ataxia Creutzfeldt Jakob disease Pathology, clinical Background: The identification of clinical and pathological forms of Creutzfeldt Jakob Disease (CJD) started with the first cases of the disease. Genetic and biomolecular prion status assessment are allowing now a better classification. Aim: To identify the clinical forms of the disease that exist in Chile, based on clinical and neuropathological data. Patients and Methods: Review of records of 40 patients with CJD in whom a complete history, clinical details and neuropathological studies were available. Clinical aspects were grouped into five categories: behavioral and cognitive changes, sleep and alertness, visual impairment, motor disturbances, myoclonus and epilepsy. The neuropathological examination in each case allowed us to evaluate the damage of 13 areas of the central nervous system. Results: Five forms of CJD were identified. The classic form was present in 28 patients (70%), the Heidenhain form was present in five (12.5%), the ataxic form in four (10%), the form with Kuru plaques in two (5%) and the Vacuolar was present in one patient (2.5%). Conclusions: The variety and forms of CJD in Chile do not differ substantially from those found abroad.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.140 n.2 20122012-02-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872012000200003es10.4067/S0034-98872012000200003
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Ataxia Creutzfeldt Jakob disease Pathology, clinical
spellingShingle	Ataxia Creutzfeldt Jakob disease Pathology, clinical Cartier R,Luis Quiroz Z,Gonzalo Leiva H,Marcelo Vergara R,Carmen Identificación clínica y patológica de las diversas formas de la enfermedad de Creutzfeldt Jakob en Chile
description	Background: The identification of clinical and pathological forms of Creutzfeldt Jakob Disease (CJD) started with the first cases of the disease. Genetic and biomolecular prion status assessment are allowing now a better classification. Aim: To identify the clinical forms of the disease that exist in Chile, based on clinical and neuropathological data. Patients and Methods: Review of records of 40 patients with CJD in whom a complete history, clinical details and neuropathological studies were available. Clinical aspects were grouped into five categories: behavioral and cognitive changes, sleep and alertness, visual impairment, motor disturbances, myoclonus and epilepsy. The neuropathological examination in each case allowed us to evaluate the damage of 13 areas of the central nervous system. Results: Five forms of CJD were identified. The classic form was present in 28 patients (70%), the Heidenhain form was present in five (12.5%), the ataxic form in four (10%), the form with Kuru plaques in two (5%) and the Vacuolar was present in one patient (2.5%). Conclusions: The variety and forms of CJD in Chile do not differ substantially from those found abroad.
author	Cartier R,Luis Quiroz Z,Gonzalo Leiva H,Marcelo Vergara R,Carmen
author_facet	Cartier R,Luis Quiroz Z,Gonzalo Leiva H,Marcelo Vergara R,Carmen
author_sort	Cartier R,Luis
title	Identificación clínica y patológica de las diversas formas de la enfermedad de Creutzfeldt Jakob en Chile
title_short	Identificación clínica y patológica de las diversas formas de la enfermedad de Creutzfeldt Jakob en Chile
title_full	Identificación clínica y patológica de las diversas formas de la enfermedad de Creutzfeldt Jakob en Chile
title_fullStr	Identificación clínica y patológica de las diversas formas de la enfermedad de Creutzfeldt Jakob en Chile
title_full_unstemmed	Identificación clínica y patológica de las diversas formas de la enfermedad de Creutzfeldt Jakob en Chile
title_sort	identificación clínica y patológica de las diversas formas de la enfermedad de creutzfeldt jakob en chile
publisher	Sociedad Médica de Santiago
publishDate	2012
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872012000200003
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Identificación clínica y patológica de las diversas formas de la enfermedad de Creutzfeldt Jakob en Chile

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