<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>0120-8748</journal-id>
<journal-title><![CDATA[Acta Neurológica Colombiana]]></journal-title>
<abbrev-journal-title><![CDATA[Acta Neurol Colomb.]]></abbrev-journal-title>
<issn>0120-8748</issn>
<publisher>
<publisher-name><![CDATA[Asociación Colombiana de Neurología]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0120-87482010000200005</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Enfermedades por priones: de la clínica a la biología molecular]]></article-title>
<article-title xml:lang="en"><![CDATA[Prion diseases: from molecular biology to clinical practice]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Villegas Lanau]]></surname>
<given-names><![CDATA[Carlos Andrés]]></given-names>
</name>
<xref ref-type="aff" rid="A01"/>
</contrib>
</contrib-group>
<aff id="A01">
<institution><![CDATA[,Universidad de Antioquia  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>06</month>
<year>2010</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>06</month>
<year>2010</year>
</pub-date>
<volume>26</volume>
<numero>2</numero>
<fpage>87</fpage>
<lpage>111</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_arttext&amp;pid=S0120-87482010000200005&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_abstract&amp;pid=S0120-87482010000200005&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_pdf&amp;pid=S0120-87482010000200005&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN Las enfermedades ocasionadas por priones son también conocidas como encefalitis espongiformes transmisibles o demencias de tipo infeccioso. En humanos las presentaciones clínicas más reconocidas son la enfermedad de Creutzfeldt-Jakob, el síndrome de Gerstmann-Sträussler-Scheinker y el insomnio fatal familiar. Son consideradas patologías poco comunes, pero los descubrimientos en biología molecular de los últimos años muestran que los mecanismos patológicos que llevan a su desarrollo pueden ser comunes a varias enfermedades neurodegenerativas, lo cual puede ampliar el espectro patológico, convirtiéndolas en alteraciones no tan infrecuentes en neurología. Esta revisión pretende dar herramientas al clínico para reconocer estas enfermedades discutiendo las presentaciones clínicas en seres humanos con sus variantes: esporádicas, infecciosas y familiares, comentando además el uso de laboratorios, criterios diagnósticos y aproximación terapéutica.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[SUMMARY Illnesses caused by prions are also known as Transmissible Spongiform Encephalopathies or infectious dementias. The most recognized clinic presentations in humans are the Creutzfeldt-Jakob disease, the Gerstmann-Sträussler-Scheinker syndrome and the Fatal Familial Insomnia. They are considered uncommon pathologies, but discoveries in molecular biology in recent years, reveal that pathological mechanisms that allow its development could be common to several neurodegenerative diseases in which the pathologic spectrum can be amplified, becoming not infrequent neurological diseases. This review gives tools to the clinical attendant in order to recognize these disorders, discussing the clinical presentations: sporadic, infectious and familiar, diagnostic work up, diagnostic criteria and therapeutic approach.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Prión]]></kwd>
<kwd lng="es"><![CDATA[biología molecular]]></kwd>
<kwd lng="es"><![CDATA[encefalitis]]></kwd>
<kwd lng="en"><![CDATA[Prions]]></kwd>
<kwd lng="en"><![CDATA[molecular biology]]></kwd>
<kwd lng="en"><![CDATA[encephalitis]]></kwd>
</kwd-group>
</article-meta>
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<front>
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<journal-id>0120-8748</journal-id>
<journal-title><![CDATA[Acta Neurológica Colombiana]]></journal-title>
<abbrev-journal-title><![CDATA[Acta Neurol Colomb.]]></abbrev-journal-title>
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<article-id>S0120-87482010000200005</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Enfermedades por priones: de la clínica a la biología molecular]]></article-title>
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<institution><![CDATA[,Universidad de Antioquia  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>06</month>
<year>2010</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>06</month>
<year>2010</year>
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<volume>26</volume>
<numero>2</numero>
<fpage>87</fpage>
<lpage>111</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_arttext&amp;pid=S0120-87482010000200005&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_abstract&amp;pid=S0120-87482010000200005&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_pdf&amp;pid=S0120-87482010000200005&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN Las enfermedades ocasionadas por priones son también conocidas como encefalitis espongiformes transmisibles o demencias de tipo infeccioso. En humanos las presentaciones clínicas más reconocidas son la enfermedad de Creutzfeldt-Jakob, el síndrome de Gerstmann-Sträussler-Scheinker y el insomnio fatal familiar. Son consideradas patologías poco comunes, pero los descubrimientos en biología molecular de los últimos años muestran que los mecanismos patológicos que llevan a su desarrollo pueden ser comunes a varias enfermedades neurodegenerativas, lo cual puede ampliar el espectro patológico, convirtiéndolas en alteraciones no tan infrecuentes en neurología. Esta revisión pretende dar herramientas al clínico para reconocer estas enfermedades discutiendo las presentaciones clínicas en seres humanos con sus variantes: esporádicas, infecciosas y familiares, comentando además el uso de laboratorios, criterios diagnósticos y aproximación terapéutica.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[SUMMARY Illnesses caused by prions are also known as Transmissible Spongiform Encephalopathies or infectious dementias. The most recognized clinic presentations in humans are the Creutzfeldt-Jakob disease, the Gerstmann-Sträussler-Scheinker syndrome and the Fatal Familial Insomnia. They are considered uncommon pathologies, but discoveries in molecular biology in recent years, reveal that pathological mechanisms that allow its development could be common to several neurodegenerative diseases in which the pathologic spectrum can be amplified, becoming not infrequent neurological diseases. This review gives tools to the clinical attendant in order to recognize these disorders, discussing the clinical presentations: sporadic, infectious and familiar, diagnostic work up, diagnostic criteria and therapeutic approach.]]></p></abstract>
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<kwd lng="en"><![CDATA[Prions]]></kwd>
<kwd lng="en"><![CDATA[molecular biology]]></kwd>
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