<?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>1665-5044</journal-id>
<journal-title><![CDATA[Revista mexicana de neurociencia]]></journal-title>
<abbrev-journal-title><![CDATA[Rev. mex. neurocienc.]]></abbrev-journal-title>
<issn>1665-5044</issn>
<publisher>
<publisher-name><![CDATA[Academia Mexicana de Neurología A.C.]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1665-50442021000400134</article-id>
<article-id pub-id-type="doi">10.24875/rmn.20000132</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Lissencephaly: Clinical and neuroimaging features in children]]></article-title>
<article-title xml:lang="es"><![CDATA[Lisencefalia: características clínicas y de neuroimagen en niños]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Lapo-Córdova]]></surname>
<given-names><![CDATA[Nathaly S.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ruiz-García]]></surname>
<given-names><![CDATA[Matilde]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Hernández-Antúnez]]></surname>
<given-names><![CDATA[Blanca G.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,National Institute of Pediatrics Department of Pediatric Neurology ]]></institution>
<addr-line><![CDATA[Mexico City ]]></addr-line>
<country>Mexico</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>08</month>
<year>2021</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>08</month>
<year>2021</year>
</pub-date>
<volume>22</volume>
<numero>4</numero>
<fpage>134</fpage>
<lpage>140</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_arttext&amp;pid=S1665-50442021000400134&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_abstract&amp;pid=S1665-50442021000400134&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_pdf&amp;pid=S1665-50442021000400134&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract  Background: The spectrum of lissencephaly (LIS) corresponds to a group of serious brain malformations in the cortex caused by a failure in neuronal migration. The spectrum includes agyria, pachygyria and subcortical band heterotopia (SBH). It has generally been divided into two categories: classic lissencephaly or type I, and cobblestone lissencephaly or type II.  Objective: The objective of the study was to describe clinical, neuroimaging, and neurophysiological features of pediatric patients with lissencephaly (LIS) type I.  Methods: Retrospective study of children with the diagnosis of LIS, who were admitted to the National Institute of Pediatrics in Mexico City from January 2009 to December 2019.  Results: We included a total of 22 patients, 15 (68%) were male. Age at diagnosis: 4 (18%) children under 1 month due to ventricular dilation on ultrasound and epileptic spasms; 13 (59%) children of 1 month-1 year due to microcephaly, drug-resistant epilepsy, and neurodevelopmental delay; 5 (22%) children over 1 year. Regarding etiology: 6 cases were due to cytomegalovirus, 1 to Zika, and 1 to microdeletion diagnosed as Miller-Dieker syndrome. All (100%) had neurodevelopmental delay, 19 (86%) intellectual disability. Epilepsy was found in 19 (86%), of these 6 had epileptic spasms, 7 had West syndrome, and 5 evolved to Lennox-Gastaut. Drug-resistant epilepsy was present in 17 (77%) patients. Regarding comorbidities: 15 (68%) had gastroesophageal reflux disease and 14 (63%) had recurrent pneumonia. Regarding neuroimaging findings, paquigiria was present in 9 (41%) children. Two children died, they had diffuse agyria.  Conclusions: LIS type I includes pathologies with a poor prognosis, manifested predominantly in the 1st year of life. All patients have delayed psychomotor development, refractory epilepsy and were associated with different comorbidities. Genetic and neuroimaging studies are important to make an accurate diagnosis, predict evolution, offer genetic counseling, and palliative treatment.]]></p></abstract>
<abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen  Antecedentes: El espectro de lisencefalia (LIS) corresponde a un grupo de graves malformaciones cerebrales en la corteza causadas por un fallo en la migración neuronal. El espectro incluye agiria, paquigiria y heterotopía de banda subcortical (SBH). Generalmente se ha dividido en dos categorías: lisencefalia clásica o tipo I y lisencefalia en empedrado o tipo II.  Objetivo: Describir las características clínicas, neuroimagen y neurofisiológicas de pacientes pediátricos con Lisencefalia tipo I.  Métodos: Estudio retrospectivo y descriptivo de pacientes con diagnóstico de lisencefalia atendidos en el Servicio de Neurología Pediátrica del Instituto Nacional de Pediatría, en la Ciudad de México, de enero de 2009 a diciembre de 2019.  Resultados: Incluimos un total de 22 pacientes, 15 (68%) eran hombres. Edad al diagnóstico: período neonatal 4 (18%) por dilatación ventricular en ultrasonido, Zika materno y espasmos epilépticos; de 1 mes a 1 año: 13 (59%) por microcefalia, epilepsia fármaco resistente y retraso del neurodesarrollo y mayores de 1 año: 5 (22%) niños. Etiología: 6 por Citomegalovirus, 1 por Zika y 1 microdeleción con síndrome de Miller Dieker. Todos tuvieron retraso del neurodesarrollo, 19 con discapacidad intelectual (3 pacientes &lt; 4 años). Epilepsia en 19 (86%), 6 tuvieron espasmos epilépticos, 7 Síndrome de West, 5 evolucionaron a Lennox Gastaut. Epilepsia fármaco resistente en 17 (77%). Comorbilidades: 14 (63%) neumonías a repetición y 15 (68%) con enfermedad por reflujo gastroesofágico. Paquigiria en 9 (41%) niños. Dos niños murieron, tenían agiria difusa.  Conclusión: Lisencefalia tipo I incluye patologías de mal pronóstico, que se manifiesta predominantemente en el primer año de vida. Todos los pacientes tienen retraso en el desarrollo psicomotor, epilepsia refractaria y se asocian a diferentes comorbilidades. Es importante el estudio genético y neuroimagen de alta resolución para realizar un diagnóstico preciso, predecir evolución, ofrecer consejo genético y tratamiento paliativo.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Lissencephaly]]></kwd>
<kwd lng="en"><![CDATA[Pachygyria]]></kwd>
<kwd lng="en"><![CDATA[Agyria]]></kwd>
<kwd lng="en"><![CDATA[Children]]></kwd>
<kwd lng="es"><![CDATA[Lisencefalia]]></kwd>
<kwd lng="es"><![CDATA[Paquigiria]]></kwd>
<kwd lng="es"><![CDATA[Agiria]]></kwd>
<kwd lng="es"><![CDATA[Niños]]></kwd>
</kwd-group>
</article-meta>
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