<?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>0034-7531</journal-id>
<journal-title><![CDATA[Revista Cubana de Pediatría]]></journal-title>
<abbrev-journal-title><![CDATA[Rev Cubana Pediatr]]></abbrev-journal-title>
<issn>0034-7531</issn>
<publisher>
<publisher-name><![CDATA[Centro Nacional de Información de Ciencias MédicasEditorial Ciencias Médicas]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0034-75312020000300012</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Diagnóstico clínico imagenológico y evolutivo de leucodistrofia, megaloencefalia y quistes subtemporales en la lactancia]]></article-title>
<article-title xml:lang="en"><![CDATA[Clinical-epidemiological and evolutive diagnosis of leukodystrophy, megalencephaly and subtemporal cysts during breastfeeding]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Vargas Díaz]]></surname>
<given-names><![CDATA[José]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Garófalo Gómez]]></surname>
<given-names><![CDATA[Nicolás]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Novoa López]]></surname>
<given-names><![CDATA[Lucía Margarita]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Bello Carbonell]]></surname>
<given-names><![CDATA[Sonia María]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Reyes Berazaín]]></surname>
<given-names><![CDATA[Adnolys]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Instituto de Neurología y Neurocirugía  ]]></institution>
<addr-line><![CDATA[La Habana ]]></addr-line>
<country>Cuba</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>09</month>
<year>2020</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>09</month>
<year>2020</year>
</pub-date>
<volume>92</volume>
<numero>3</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_arttext&amp;pid=S0034-75312020000300012&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_abstract&amp;pid=S0034-75312020000300012&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_pdf&amp;pid=S0034-75312020000300012&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  Las leucodistrofias primarias son un grupo de trastornos hereditarios que afectan la sustancia blanca cerebral de forma predominante. El término leucodistrofias primarias unifica las enfermedades que afectan las células gliales con compromiso de la mielina y el axón de base genética. Es un grupo de entidades, más de 30 en la actualidad, en expansión gracias a los avances en resonancia magnética y en genética.  Objetivo:  Contribuir al conocimiento de esta rara enfermedad con énfasis en la utilidad de su conocimiento clínico y en los estudios de imágenes evolutivos para el diagnóstico.  Presentación del caso:  Paciente con macrocranea detectada por la familia a los tres meses de nacida, al inicio refirieron que la circunferencia cefálica creció rápidamente y a partir del segundo semestre de vida el crecimiento se hizo más lento. La valoración clínica al año de edad comprobó una discreta dificultad para el control cefálico, intentaba dar pasos con apoyo, lenguaje de 3 a 4 palabras y ninguna otra afectación del neurodesarrollo. Al examen físico, una circunferencia cefálica de 55 cm (por encima de dos desviaciones estándar para la edad cronológica y sexo).  Conclusiones:  La evolución clínica favorable conjuntamente con el patrón de la resonancia magnética cerebral, que inicialmente mostró afectación de la sustancia blanca compatible con leucodistrofia inespecífica y en estudios evolutivos detecta presencia de quistes subtemporales, permitieron el diagnóstico de esta rara enfermedad infantil. La paciente recibió tratamiento sintomático para la espasticidad, apoyo pedagógico y control de sus crisis epilépticas.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction: Primary leukodistrophies are a group of hereditary disorders that affect in a predominant way the white substance of the brain. The term ´´primary leukodistrophies´´ unifies the diseases that affect the glial cells compromising myelin and the genetic based axon. They are a group of entities, more tan 30 nowadays, which are expanding due to the advances in magnetic resonance and genetics.  Objective:  To contribute to the understanding of this rare disease with emphasis in the usefulness of its clinical knowledge and in the evolutive studies of images for diagnosis.  Case presentation:  Female patient with macrocranea detected by the family at 3 months old. At the beginning, the family referred that the cephalic circumference grew quickly and from the second semester of life growing process was slower. The clinical assessment at first year of life proved a discreet difficulty for cephalic control; the patient tried to do support footsteps, and had a language of 3 to 4 words without any other affectation in the neurodevelopment. In the physical examination, she presented a cephalic circumference of 55 cm (higher than the two stantard deviations for the cronological age and sex).  Conclusiones:  The clinical evolution was favorable jointly with the pattern of brain magnetic resonance that initially showed affectation in the white substance compatible with inespecific leukodistrophy and in the evolutive studies it was detected the presence of subtemporal cysts which allowed to diagnose this rare children disease. The patient received symptomatic treatment for spasticity, pedagogical support and control of the epileptic crisis.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[leucodistrofia]]></kwd>
<kwd lng="es"><![CDATA[macrocranea]]></kwd>
<kwd lng="es"><![CDATA[leucoencefalopatías]]></kwd>
<kwd lng="es"><![CDATA[leucoencefalopatía megaloencefálica con quistes (MLC)]]></kwd>
<kwd lng="en"><![CDATA[leukodistrophy]]></kwd>
<kwd lng="en"><![CDATA[macrocranea]]></kwd>
<kwd lng="en"><![CDATA[leukoencephalopathies]]></kwd>
<kwd lng="en"><![CDATA[megaloencephalic leukoencephalopathy with cysts (MLC)]]></kwd>
</kwd-group>
</article-meta>
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