<?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>2664-3243</journal-id>
<journal-title><![CDATA[Vive Revista de Salud]]></journal-title>
<abbrev-journal-title><![CDATA[Vive Rev. Salud]]></abbrev-journal-title>
<issn>2664-3243</issn>
<publisher>
<publisher-name><![CDATA[CET-BOLIVIA]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2664-32432025000301223</article-id>
<article-id pub-id-type="doi">10.33996/revistavive.v8i24.445</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Más allá del parkinson: ELA con fenotipo extrapiramidal no guam, un desafío clínico progresivo]]></article-title>
<article-title xml:lang="en"><![CDATA[Beyond Parkinson's: ALS with non-Guam extrapyramidal phenotype, a progressive clinical challenge]]></article-title>
<article-title xml:lang="pt"><![CDATA[Além do Parkinson: ELA com fenótipo extrapiramidal não-Guam, um desafio clínico progressivo]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Bombón Chico]]></surname>
<given-names><![CDATA[José Enrique]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Hugo Merino]]></surname>
<given-names><![CDATA[Gabriel Aníbal]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Domínguez Noboa]]></surname>
<given-names><![CDATA[Geoandy Elizabeth]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Fonseca Vaca]]></surname>
<given-names><![CDATA[Juan Daniel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Uruchima Romero]]></surname>
<given-names><![CDATA[Lisseth Paola]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cantos Jiménez]]></surname>
<given-names><![CDATA[Mélany Germania]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital José Carrasco Arteaga del Instituto Ecuatoriano de Seguridad Social  ]]></institution>
<addr-line><![CDATA[Cuenca ]]></addr-line>
<country>Ecuador</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>12</month>
<year>2025</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>12</month>
<year>2025</year>
</pub-date>
<volume>8</volume>
<numero>24</numero>
<fpage>1223</fpage>
<lpage>1234</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.bo/scielo.php?script=sci_arttext&amp;pid=S2664-32432025000301223&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.bo/scielo.php?script=sci_abstract&amp;pid=S2664-32432025000301223&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.bo/scielo.php?script=sci_pdf&amp;pid=S2664-32432025000301223&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN La esclerosis lateral amiotrófica (ELA) es una afección neurodegenerativa progresiva que se caracteriza por la degeneración de las neuronas motoras superiores e inferiores. Si bien su manifestación convencional es de naturaleza piramidal, existen fenotipos extrapiramidales atípicos que amplían su alcance clínico y desafían los parámetros diagnósticos establecidos. Presentamos el caso de una paciente femenina de 64 años diagnosticada inicialmente con trastorno parkinsoniano, con hallazgos al examen físico compatibles con: rigidez, bradicinesia, posturas distónicas y fasciculaciones linguales, todas ellas insensibles al tratamiento con levodopa. Las evaluaciones electromiográficas revelaron una denervación tanto activa como crónica, mientras que las imágenes de resonancia magnética del cerebro indicaron una atrofia cortical generalizada y características morfológicas consistentes con el &#8220;signo del colibrí&#8221; y el &#8220;signo de morning glory&#8221;, lo que sugiere la participación del mesencéfalo y la protuberancia sin marcadores distintivos de parkinsonismo atípico. La presencia de signos en las neuronas motoras superiores e inferiores, junto con los síntomas extrapiramidales, favorecieron el diagnóstico de la ELA extrapiramidal en Guam. El tratamiento incluyó riluzol, biperideno y un enfoque de rehabilitación multidisciplinario, con el objetivo de mantener la funcionalidad y mejorar la calidad de vida. Este caso subraya la necesidad de una metodología clínica holística, en la que la integración de los hallazgos clínicos, los datos electromiográficos y las neuroimágenes sea crucial para identificar las manifestaciones no tradicionales de la ELA. El reconocimiento de estos fenotipos es fundamental para refinar los criterios diagnósticos y optimizar las estrategias terapéuticas en una enfermedad cuyo espectro neurodegenerativo sigue evolucionando.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by the degeneration of upper and lower motor neurons. While its conventional manifestation is pyramidal in nature, atypical extrapyramidal phenotypes exist that broaden its clinical scope and challenge established diagnostic parameters. We present the case of a 64-year-old female patient initially diagnosed with parkinsonian disorder, with physical examination findings consistent with rigidity, bradykinesia, dystonic postures, and tongue fasciculations, all unresponsive to levodopa treatment. Electromyographic evaluations revealed both active and chronic denervation, while brain magnetic resonance imaging indicated generalized cortical atrophy and morphological features consistent with the "hummingbird sign" and "morning glory sign," suggesting involvement of the midbrain and pons without distinctive markers of atypical parkinsonism. The presence of signs in both upper and lower motor neurons, along with extrapyramidal symptoms, supported the diagnosis of extrapyramidal ALS in Guam. Treatment included riluzole, biperiden, and a multidisciplinary rehabilitation approach, with the goal of maintaining function and improving quality of life. This case underscores the need for a holistic clinical methodology, in which the integration of clinical findings, electromyographic data, and neuroimaging is crucial for identifying non-traditional manifestations of ALS. Recognizing these phenotypes is fundamental for refining diagnostic criteria and optimizing therapeutic strategies in a disease whose neurodegenerative spectrum continues to evolve.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[RESUMO A esclerose lateral amiotrófica (ELA) é uma doença neurodegenerativa progressiva caracterizada pela degeneração dos neurônios motores superiores e inferiores. Embora sua manifestação convencional seja piramidal, existem fenótipos extrapiramidais atípicos que ampliam seu escopo clínico e desafiam os parâmetros diagnósticos estabelecidos. Apresentamos o caso de uma paciente de 64 anos inicialmente diagnosticada com transtorno parkinsoniano, com achados no exame físico compatíveis com rigidez, bradicinesia, posturas distônicas e fasciculações da língua, todos sem resposta ao tratamento com levodopa. Avaliações eletromiográficas revelaram desnervação ativa e crônica, enquanto a ressonância magnética cerebral indicou atrofia cortical generalizada e características morfológicas compatíveis com o "sinal do beija-flor" e o "sinal da glória da manhã", sugerindo envolvimento do mesencéfalo e da ponte sem marcadores distintivos de parkinsonismo atípico. A presença de sinais tanto nos neurônios motores superiores quanto nos inferiores, juntamente com sintomas extrapiramidais, corroborou o diagnóstico de ELA extrapiramidal em Guam. O tratamento incluiu riluzol, biperideno e uma abordagem de reabilitação multidisciplinar, com o objetivo de manter a função e melhorar a qualidade de vida. Este caso ressalta a necessidade de uma metodologia clínica holística, na qual a integração de achados clínicos, dados eletromiográficos e neuroimagem é crucial para a identificação de manifestações não tradicionais da ELA. O reconhecimento desses fenótipos é fundamental para refinar os critérios diagnósticos e otimizar as estratégias terapêuticas em uma doença cujo espectro neurodegenerativo continua a evoluir.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Esclerosis amiotrófica lateral]]></kwd>
<kwd lng="es"><![CDATA[Trastornos parkinsonianos]]></kwd>
<kwd lng="es"><![CDATA[Guam]]></kwd>
<kwd lng="es"><![CDATA[Enfermedad de neurona motora]]></kwd>
<kwd lng="en"><![CDATA[Amyotrophic lateral sclerosis]]></kwd>
<kwd lng="en"><![CDATA[Parkinsonian disorders]]></kwd>
<kwd lng="en"><![CDATA[Guam]]></kwd>
<kwd lng="en"><![CDATA[Motor neuron disease]]></kwd>
<kwd lng="pt"><![CDATA[Esclerose lateral amiotrófica]]></kwd>
<kwd lng="pt"><![CDATA[Doenças parkinsonianas]]></kwd>
<kwd lng="pt"><![CDATA[Guam]]></kwd>
<kwd lng="pt"><![CDATA[Doença do neurônio motor]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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