{"id":41491,"date":"2026-02-09T11:00:00","date_gmt":"2026-02-09T14:00:00","guid":{"rendered":"https:\/\/www.grupomedcof.com.br\/blog\/?p=41491"},"modified":"2026-02-03T17:36:50","modified_gmt":"2026-02-03T20:36:50","slug":"malformacoes-do-sistema-nervoso-central","status":"publish","type":"post","link":"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/","title":{"rendered":"Como as malforma\u00e7\u00f5es do Sistema Nervoso Central e Perif\u00e9rico se manifestam?"},"content":{"rendered":"<div id=\"ez-toc-container\" class=\"ez-toc-v2_0_74 counter-hierarchy ez-toc-counter ez-toc-transparent ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">Sum\u00e1rio<\/p>\n<span class=\"ez-toc-title-toggle\"><a href=\"#\" class=\"ez-toc-pull-right ez-toc-btn ez-toc-btn-xs ez-toc-btn-default ez-toc-toggle\" aria-label=\"Alternar tabela de conte\u00fado\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Toggle<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #999;color:#999\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewBox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 16h2v2H4v-2zm16 0H8v2h12v-2z\" fill=\"currentColor\"><\/path><\/svg><svg style=\"fill: #999;color:#999\" class=\"arrow-unsorted-368013\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"10px\" height=\"10px\" viewBox=\"0 0 24 24\" version=\"1.2\" baseProfile=\"tiny\"><path d=\"M18.2 9.3l-6.2-6.3-6.2 6.3c-.2.2-.3.4-.3.7s.1.5.3.7c.2.2.4.3.7.3h11c.3 0 .5-.1.7-.3.2-.2.3-.5.3-.7s-.1-.5-.3-.7zM5.8 14.7l6.2 6.3 6.2-6.3c.2-.2.3-.5.3-.7s-.1-.5-.3-.7c-.2-.2-.4-.3-.7-.3h-11c-.3 0-.5.1-.7.3-.2.2-.3.5-.3.7s.1.5.3.7z\"\/><\/svg><\/span><\/span><\/span><\/a><\/span><\/div>\n<nav><ul class='ez-toc-list ez-toc-list-level-1 ' ><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-1\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Disrafismos_Defeitos_do_Fechamento_do_Tubo_Neural\" >Disrafismos: Defeitos do Fechamento do Tubo Neural<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Classificacao_dos_Disrafismos_Espinhais\" >Classifica\u00e7\u00e3o dos Disrafismos Espinhais<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Disrafismos_Cranianos\" >Disrafismos Cranianos<\/a><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Malformacoes_de_Fossa_Posterior\" >Malforma\u00e7\u00f5es de Fossa Posterior<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Malformacoes_de_Chiari\" >Malforma\u00e7\u00f5es de Chiari<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Malformacao_de_Dandy-Walker\" >Malforma\u00e7\u00e3o de Dandy-Walker<\/a><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Disturbios_da_Migracao_e_Organizacao_Cortical\" >Dist\u00farbios da Migra\u00e7\u00e3o e Organiza\u00e7\u00e3o Cortical<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Lisencefalia_%E2%80%9CCerebro_Liso%E2%80%9D\" >Lisencefalia (&#8220;C\u00e9rebro Liso&#8221;)<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-9\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Heterotopias\" >Heterotopias<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-10\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Esquizencefalia\" >Esquizencefalia<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-11\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Displasia_Cortical_Focal\" >Displasia Cortical Focal<\/a><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-12\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Malformacoes_do_Prosencefalo_Linha_Media\" >Malforma\u00e7\u00f5es do Prosenc\u00e9falo (Linha M\u00e9dia)<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-13\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Holoprosencefalia\" >Holoprosencefalia<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-14\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Displasia_Septo-Optica_Sd_de_Morsier\" >Displasia Septo-\u00d3ptica (Sd. de Morsier)<\/a><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-15\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Sindromes_do_Tronco_Encefalico_Nervos_Cranianos\" >S\u00edndromes do Tronco Encef\u00e1lico (Nervos Cranianos)<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-16\" href=\"https:\/\/www.grupomedcof.com.br\/blog\/malformacoes-do-sistema-nervoso-central\/#Conquiste_a_aprovacao_e_acompanhe_mais_noticias\" >Conquiste a aprova\u00e7\u00e3o e acompanhe mais not\u00edcias!<\/a><\/li><\/ul><\/nav><\/div>\n\n<p>As <strong>malforma\u00e7\u00f5es neurol\u00f3gicas <\/strong>s\u00e3o &#8220;erros de constru\u00e7\u00e3o&#8221; que ocorrem em diferentes etapas do desenvolvimento embrion\u00e1rio: neurula\u00e7\u00e3o (fechamento do tubo), prolifera\u00e7\u00e3o neuronal, migra\u00e7\u00e3o ou organiza\u00e7\u00e3o cortical.\u00a0<\/p>\n\n\n\n<figure class=\"wp-block-image size-full\"><img loading=\"lazy\" decoding=\"async\" width=\"736\" height=\"981\" src=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-15.jpeg\" alt=\"\" class=\"wp-image-41492\" srcset=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-15.jpeg 736w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-15-225x300.jpeg 225w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-15-315x420.jpeg 315w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-15-150x200.jpeg 150w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-15-300x400.jpeg 300w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-15-696x928.jpeg 696w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-15-640x853.jpeg 640w\" sizes=\"auto, (max-width: 736px) 100vw, 736px\" \/><figcaption class=\"wp-element-caption\">Fonte: <a href=\"https:\/\/pin.it\/3KB7cztEt\">https:\/\/pin.it\/3KB7cztEt<\/a>\u00a0<\/figcaption><\/figure>\n\n\n\n<h2 class=\"wp-block-heading\" id=\"h-disrafismos-defeitos-do-fechamento-do-tubo-neural\"><span class=\"ez-toc-section\" id=\"Disrafismos_Defeitos_do_Fechamento_do_Tubo_Neural\"><\/span><strong>Disrafismos: Defeitos do Fechamento do Tubo Neural<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p>O termo <strong>Disrafismo<\/strong> refere-se \u00e0 falha no fechamento \u00f3sseo posterior do esqueleto axial (seja craniano ou espinhal). O uso de \u00e1cido f\u00f3lico no per\u00edodo periconcepcional \u00e9 o principal fator protetor.<\/p>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-classificacao-dos-disrafismos-espinhais\"><span class=\"ez-toc-section\" id=\"Classificacao_dos_Disrafismos_Espinhais\"><\/span><strong>Classifica\u00e7\u00e3o dos Disrafismos Espinhais<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>A distin\u00e7\u00e3o crucial \u00e9 a presen\u00e7a ou n\u00e3o de pele recobrindo a les\u00e3o:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Disrafismo Aberto (N\u00e3o recoberto por pele):<\/strong>\n<ul class=\"wp-block-list\">\n<li><strong>Mielomeningocele (MMC):<\/strong> \u00c9 o defeito mais comum. O saco herni\u00e1rio cont\u00e9m meninges + medula\/ra\u00edzes nervosas. 80% s\u00e3o lombossacrais. Est\u00e1 quase invariavelmente associada a Chiari II.<\/li>\n\n\n\n<li><strong>Mielocele:<\/strong> Apenas a medula hernia e fica exposta, sem o recobrimento men\u00edngeo c\u00edstico cl\u00e1ssico.<\/li>\n\n\n\n<li><strong>Meningocele:<\/strong> O saco cont\u00e9m apenas meninges e l\u00edquor, sem tecido nervoso.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Disrafismo Fechado (Oculto):<\/strong>\n<ul class=\"wp-block-list\">\n<li>A les\u00e3o \u00e9 recoberta por pele. Pode haver estigmas cut\u00e2neos (tufos pilosos, hemangiomas, fossetas) indicando a malforma\u00e7\u00e3o subjacente.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<p><img loading=\"lazy\" decoding=\"async\" width=\"602\" height=\"343\" src=\"blob:https:\/\/www.grupomedcof.com.br\/dbab6875-3060-453b-8244-c6b3e9798934\"><\/p>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-disrafismos-cranianos\"><span class=\"ez-toc-section\" id=\"Disrafismos_Cranianos\"><\/span><strong>Disrafismos Cranianos<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Encefalocele:<\/strong> Falha \u00f3ssea com exterioriza\u00e7\u00e3o de meninges e tecido encef\u00e1lico.<\/li>\n\n\n\n<li><strong>Meningocele Craniana:<\/strong> Apenas meninges se exteriorizam.<\/li>\n<\/ul>\n\n\n\n<h2 class=\"wp-block-heading\" id=\"h-malformacoes-de-fossa-posterior\"><span class=\"ez-toc-section\" id=\"Malformacoes_de_Fossa_Posterior\"><\/span><strong>Malforma\u00e7\u00f5es de Fossa Posterior<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p>Aqui, a anatomia radiol\u00f3gica \u00e9 decisiva.<\/p>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-malformacoes-de-chiari\"><span class=\"ez-toc-section\" id=\"Malformacoes_de_Chiari\"><\/span><strong>Malforma\u00e7\u00f5es de Chiari<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>S\u00e3o anomalias da jun\u00e7\u00e3o craniocervical. A classifica\u00e7\u00e3o detalhada inclui:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Chiari I:<\/strong> Hernia\u00e7\u00e3o apenas das <strong>tonsilas cerebelares<\/strong> pelo forame magno. Pode ser assintom\u00e1tico ou gerar cefaleia tuss\u00edgena\/siringomielia. Tratamento: Descompress\u00e3o se sintom\u00e1tico.<\/li>\n<\/ul>\n\n\n\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"1024\" height=\"416\" src=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-41-1024x416.png\" alt=\"\" class=\"wp-image-41493\" srcset=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-41-1024x416.png 1024w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-41-300x122.png 300w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-41-768x312.png 768w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-41-1034x420.png 1034w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-41-150x61.png 150w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-41-696x283.png 696w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-41-1068x434.png 1068w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-41.png 1128w\" sizes=\"auto, (max-width: 1024px) 100vw, 1024px\" \/><figcaption class=\"wp-element-caption\">Malforma\u00e7\u00e3o de Chiari do tipo 1. Note a presen\u00e7a de hernia\u00e7\u00e3o tonsilar e de dilata\u00e7\u00e3o secund\u00e1ria do canal central da medula (siringomielia). Fonte: Acervo de Aulas do Grupo MedCof.\u00a0<\/figcaption><\/figure>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Chiari II (Arnold-Chiari):<\/strong> Hernia\u00e7\u00e3o das tonsilas <strong>+ V\u00e9rmis Cerebelar<\/strong> + Tronco encef\u00e1lico. <strong>Associa\u00e7\u00e3o:<\/strong> Quase 100% associado \u00e0 Mielomeningocele lombossacral. Requer corre\u00e7\u00e3o cir\u00fargica precoce (intra\u00fatero ou &lt;72h).<\/li>\n\n\n\n<li><strong>Chiari III:<\/strong> Semelhante ao II, mas com <strong>encefalocele<\/strong> occipital\/cervical alta.<\/li>\n\n\n\n<li><strong>Chiari IV:<\/strong> Hipoplasia cerebelar grave <em>sem<\/em> hernia\u00e7\u00e3o.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-malformacao-de-dandy-walker\"><span class=\"ez-toc-section\" id=\"Malformacao_de_Dandy-Walker\"><\/span><strong>Malforma\u00e7\u00e3o de Dandy-Walker<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>A tr\u00edade diagn\u00f3stica cl\u00e1ssica na RM \u00e9:<\/p>\n\n\n\n<ol class=\"wp-block-list\">\n<li>Hipoplasia ou agenesia do v\u00e9rmis cerebelar (com rota\u00e7\u00e3o cef\u00e1lica do remanescente).<\/li>\n\n\n\n<li>Dilata\u00e7\u00e3o c\u00edstica do IV ventr\u00edculo.<\/li>\n\n\n\n<li>Aumento volum\u00e9trico da fossa posterior.<\/li>\n<\/ol>\n\n\n\n<figure class=\"wp-block-image size-full\"><img loading=\"lazy\" decoding=\"async\" width=\"997\" height=\"332\" src=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-42.png\" alt=\"\" class=\"wp-image-41494\" srcset=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-42.png 997w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-42-300x100.png 300w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-42-768x256.png 768w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-42-150x50.png 150w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-42-696x232.png 696w\" sizes=\"auto, (max-width: 997px) 100vw, 997px\" \/><figcaption class=\"wp-element-caption\">Paciente com Malforma\u00e7\u00e3o de Dandy-Walker. Na primeira imagem, corte axial da fossa posterior mostrando aumento das dimens\u00f5es da fossa posterior e agenesia cerebelar. Na segunda imagem, mostra a repercuss\u00e3o com hidrocefalia com o aumento dos ventr\u00edculos laterais (presente na maioria dos casos de Dandy-Walker). Na terceira imagem temos um corte sagital mostrando a dilata\u00e7\u00e3o do sistema ventricular, hidrocefalia e aumento das dimens\u00f5es da fossa posterior. Fonte: Acervo de aulas do Grupo MedCof.\u00a0<\/figcaption><\/figure>\n\n\n\n<h2 class=\"wp-block-heading\" id=\"h-disturbios-da-migracao-e-organizacao-cortical\"><span class=\"ez-toc-section\" id=\"Disturbios_da_Migracao_e_Organizacao_Cortical\"><\/span><strong>Dist\u00farbios da Migra\u00e7\u00e3o e Organiza\u00e7\u00e3o Cortical<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p>Estas s\u00e3o causas frequentes de <strong>epilepsia refrat\u00e1ria<\/strong> e atraso no desenvolvimento. Ocorrem quando os neur\u00f4nios n\u00e3o chegam ao local correto ou n\u00e3o se organizam em camadas.<\/p>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-lisencefalia-cerebro-liso\"><span class=\"ez-toc-section\" id=\"Lisencefalia_%E2%80%9CCerebro_Liso%E2%80%9D\"><\/span><strong>Lisencefalia (&#8220;C\u00e9rebro Liso&#8221;)<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>Falha grave na migra\u00e7\u00e3o. O c\u00f3rtex fica espesso e com poucas ou nenhuma circunvolu\u00e7\u00e3o.<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Agiria:<\/strong> Aus\u00eancia total de giros.<\/li>\n\n\n\n<li><strong>Paquigiria:<\/strong> Giros poucos, largos e rasos.<\/li>\n\n\n\n<li><strong>Gen\u00e9tica:<\/strong> Gene <em>LIS1<\/em> (PAFAH1B1) em 60% dos casos.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-heterotopias\"><span class=\"ez-toc-section\" id=\"Heterotopias\"><\/span><strong>Heterotopias<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>Neur\u00f4nios que interromperam a migra\u00e7\u00e3o e ficaram &#8220;no meio do caminho&#8221;.<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Nodular Periventricular:<\/strong> N\u00f3dulos de subst\u00e2ncia cinzenta colados \u00e0 parede dos ventr\u00edculos (\u00e9 a forma mais comum).<\/li>\n<\/ul>\n\n\n\n<figure class=\"wp-block-image size-full\"><img loading=\"lazy\" decoding=\"async\" width=\"306\" height=\"320\" src=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-43.png\" alt=\"\" class=\"wp-image-41495\" srcset=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-43.png 306w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-43-287x300.png 287w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-43-150x157.png 150w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-43-300x314.png 300w\" sizes=\"auto, (max-width: 306px) 100vw, 306px\" \/><figcaption class=\"wp-element-caption\">Fonte: Acervo de aulas do Grupo MedCof.\u00a0<\/figcaption><\/figure>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Em Banda (C\u00f3rtex Duplo):<\/strong> Uma faixa de subst\u00e2ncia cinzenta no meio da subst\u00e2ncia branca, entre o ventr\u00edculo e o c\u00f3rtex.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-esquizencefalia\"><span class=\"ez-toc-section\" id=\"Esquizencefalia\"><\/span><strong>Esquizencefalia<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>\u00c9 uma <strong>fenda<\/strong> revestida por subst\u00e2ncia cinzenta (polimicrogiria) que comunica o espa\u00e7o subaracnoideo com o ventr\u00edculo.<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>L\u00e1bios Fechados (Tipo I):<\/strong> As paredes da fenda se tocam.<\/li>\n\n\n\n<li><strong>L\u00e1bios Abertos (Tipo II):<\/strong> H\u00e1 um canal de l\u00edquor comunicando as cavidades.<\/li>\n\n\n\n<li><strong>Diferencial com Porencefalia:<\/strong> Na porencefalia (destrui\u00e7\u00e3o tecidual), a cavidade \u00e9 revestida por subst\u00e2ncia branca\/gliose, <strong>n\u00e3o<\/strong> por subst\u00e2ncia cinzenta.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-displasia-cortical-focal\"><span class=\"ez-toc-section\" id=\"Displasia_Cortical_Focal\"><\/span><strong>Displasia Cortical Focal<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>Principal causa de epilepsia focal estrutural em crian\u00e7as. Classifica\u00e7\u00e3o atual:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Tipo I (Leve):<\/strong> Anormalidade na arquitetura (lamina\u00e7\u00e3o) cortical.<\/li>\n\n\n\n<li><strong>Tipo II (Grave\/Taylor):<\/strong> Presen\u00e7a de c\u00e9lulas anormais (neur\u00f4nios dism\u00f3rficos ou c\u00e9lulas em bal\u00e3o).\n<ul class=\"wp-block-list\">\n<li><em>Sinal na RM:<\/em> &#8220;Transmantle sign&#8221; (hipersinal em T2\/FLAIR que se estende do c\u00f3rtex at\u00e9 o ventr\u00edculo). Tem excelente resposta cir\u00fargica.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Tipo III (Associada):<\/strong> DCF junto com outra les\u00e3o (ex: Esclerose Mesial Temporal, Tumor Glioneural ou Malforma\u00e7\u00e3o Vascular).<\/li>\n<\/ul>\n\n\n\n<h2 class=\"wp-block-heading\" id=\"h-malformacoes-do-prosencefalo-linha-media\"><span class=\"ez-toc-section\" id=\"Malformacoes_do_Prosencefalo_Linha_Media\"><\/span><strong>Malforma\u00e7\u00f5es do Prosenc\u00e9falo (Linha M\u00e9dia)<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-holoprosencefalia\"><span class=\"ez-toc-section\" id=\"Holoprosencefalia\"><\/span><strong>Holoprosencefalia<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>Falha na clivagem (separa\u00e7\u00e3o) dos hemisf\u00e9rios cerebrais. Pode ser Alobar (fus\u00e3o total &#8211; mais grave), Semilobar ou Lobar. Frequentemente associada a defeitos faciais (ciclopia, fenda labial central).<\/p>\n\n\n\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"1024\" height=\"286\" src=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-44-1024x286.png\" alt=\"\" class=\"wp-image-41496\" srcset=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-44-1024x286.png 1024w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-44-300x84.png 300w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-44-768x214.png 768w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-44-150x42.png 150w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-44-696x194.png 696w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-44.png 1060w\" sizes=\"auto, (max-width: 1024px) 100vw, 1024px\" \/><figcaption class=\"wp-element-caption\">Tipos de holoprosencefalia. Fonte: Acervo de aulas do Grupo MedCof.<\/figcaption><\/figure>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-displasia-septo-optica-sd-de-morsier\"><span class=\"ez-toc-section\" id=\"Displasia_Septo-Optica_Sd_de_Morsier\"><\/span><strong>Displasia Septo-\u00d3ptica (Sd. de Morsier)<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p>Tr\u00edade (30% dos casos completos):<\/p>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Hipoplasia do Nervo \u00d3ptico.<\/strong><\/li>\n\n\n\n<li><strong>Disfun\u00e7\u00e3o Hipot\u00e1lamo-Hipofis\u00e1ria<\/strong> (defici\u00eancia de GH, hipotireoidismo).<\/li>\n\n\n\n<li><strong>Defeitos de Linha M\u00e9dia<\/strong> (aus\u00eancia de septo pel\u00facido e corpo caloso).<\/li>\n<\/ol>\n\n\n\n<figure class=\"wp-block-image size-full\"><img loading=\"lazy\" decoding=\"async\" width=\"759\" height=\"372\" src=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-45.png\" alt=\"\" class=\"wp-image-41497\" srcset=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-45.png 759w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-45-300x147.png 300w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-45-150x74.png 150w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-45-696x341.png 696w\" sizes=\"auto, (max-width: 759px) 100vw, 759px\" \/><figcaption class=\"wp-element-caption\">Imagens de RNM de uma crian\u00e7a demonstra aus\u00eancia do septo pel\u00facido (imagem axial) e um quiasma \u00f3ptico que est\u00e1 significativamente reduzido em tamanho (imagem coronal). Fonte: Acervo de aulas do Grupo MedCof.\u00a0<\/figcaption><\/figure>\n\n\n\n<h2 class=\"wp-block-heading\" id=\"h-sindromes-do-tronco-encefalico-nervos-cranianos\"><span class=\"ez-toc-section\" id=\"Sindromes_do_Tronco_Encefalico_Nervos_Cranianos\"><\/span><strong>S\u00edndromes do Tronco Encef\u00e1lico (Nervos Cranianos)<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>S\u00edndrome de Moebius:<\/strong> Agenesia dos n\u00facleos dos pares cranianos VI (Abducente) e VII (Facial).\n<ul class=\"wp-block-list\">\n<li><em>Cl\u00ednica:<\/em> &#8220;F\u00e1cies em m\u00e1scara&#8221; (paralisia facial) + Estrabismo convergente (n\u00e3o abduz o olho).<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<figure class=\"wp-block-image size-full\"><img loading=\"lazy\" decoding=\"async\" width=\"496\" height=\"484\" src=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-46.png\" alt=\"\" class=\"wp-image-41498\" srcset=\"https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-46.png 496w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-46-300x293.png 300w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-46-430x420.png 430w, https:\/\/www.grupomedcof.com.br\/blog\/wp-content\/uploads\/2026\/02\/image-46-150x146.png 150w\" sizes=\"auto, (max-width: 496px) 100vw, 496px\" \/><figcaption class=\"wp-element-caption\">1. Paciente com s\u00edndrome de Moebius em posi\u00e7\u00e3o de relaxamento muscular, evidenciando sinais de paralisia facial, com desvio da rima para a direita e apagamento de sulco nasolabial \u00e0 esquerda. 2. Sinal de Bell bilateral: note o fechamento ocular incompleto bilateral, mostrando o acometimento com fraqueza bilateral da face. A e B. planos axial e sagital em sequ\u00eancias T2; N\u00e3o \u00e9 visualizado os VII pares no ter\u00e7o anterior superior da conduta auditiva interna, o c\u00edrculo em B marca a topografi a habitual do nervo. C e D. VII par craniano \u00e9 observado adequadamente no paciente sem anormalidades (fl echas azuis). Fonte: Acervo de aulas do Grupo MedCof.\u00a0<\/figcaption><\/figure>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>S\u00edndrome de Duane:<\/strong> Agenesia isolada do VI par (Abducente) com inerva\u00e7\u00e3o an\u00f4mala do Reto Lateral pelo III par.\n<ul class=\"wp-block-list\">\n<li><em>Cl\u00ednica:<\/em> Retra\u00e7\u00e3o do globo ocular e estreitamento da fenda palpebral na tentativa de adu\u00e7\u00e3o.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h2 class=\"wp-block-heading\" id=\"h-conquiste-a-aprovacao-e-acompanhe-mais-noticias\"><span class=\"ez-toc-section\" id=\"Conquiste_a_aprovacao_e_acompanhe_mais_noticias\"><\/span><strong>Conquiste a aprova\u00e7\u00e3o e acompanhe mais not\u00edcias!<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p>Gostou do conte\u00fado? Continue acompanhando nosso blog para ficar por dentro das principais&nbsp;<strong>not\u00edcias sobre&nbsp;<a href=\"https:\/\/www.grupomedcof.com.br\/home\/\" target=\"_blank\" rel=\"noreferrer noopener\">resid\u00eancia m\u00e9dica,<\/a>&nbsp;editais e&nbsp;<a href=\"https:\/\/www.grupomedcof.com.br\/blog\/presenca-da-medcof-nas-aprovacoes-em-residencia-medica-no-brasil\/\" target=\"_blank\" rel=\"noreferrer noopener\">oportunidades que podem transformar sua carreira!<\/a><\/strong><\/p>\n\n\n\n<figure class=\"wp-block-embed is-type-video is-provider-youtube wp-block-embed-youtube wp-embed-aspect-16-9 wp-has-aspect-ratio\"><div class=\"wp-block-embed__wrapper\">\n<div class=\"youtube-embed\" data-video_id=\"hyOMy6Qt86k\"><iframe loading=\"lazy\" title=\"Malforma\u00e7\u00f5es Cong\u00eanitas do Sistema Nervoso: Sintomas e Diagn\u00f3stico. Com Dr. Vin\u00edcius Braga\" width=\"696\" height=\"392\" src=\"https:\/\/www.youtube.com\/embed\/hyOMy6Qt86k?feature=oembed&#038;enablejsapi=1\" frameborder=\"0\" allow=\"accelerometer; autoplay; clipboard-write; encrypted-media; gyroscope; picture-in-picture; web-share\" referrerpolicy=\"strict-origin-when-cross-origin\" allowfullscreen><\/iframe><\/div>\n<\/div><\/figure>\n","protected":false},"excerpt":{"rendered":"<p>As malforma\u00e7\u00f5es neurol\u00f3gicas s\u00e3o &#8220;erros de constru\u00e7\u00e3o&#8221; que ocorrem em diferentes etapas do desenvolvimento embrion\u00e1rio. Confira j\u00e1!<\/p>\n","protected":false},"author":21,"featured_media":41492,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[190,120],"tags":[3451],"ppma_author":[3617],"class_list":{"0":"post-41491","1":"post","2":"type-post","3":"status-publish","4":"format-standard","5":"has-post-thumbnail","7":"category-neurologia","8":"category-pediatria","9":"tag-neuroped"},"yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v27.6 (Yoast SEO v27.6) - https:\/\/yoast.com\/product\/yoast-seo-premium-wordpress\/ -->\n<title>Como as malforma\u00e7\u00f5es do Sistema Nervoso Central e Perif\u00e9rico se manifestam? - Blog de Medcof: conte\u00fados, editais, revalida e mais!<\/title>\n<meta name=\"description\" content=\"As malforma\u00e7\u00f5es neurol\u00f3gicas s\u00e3o &quot;erros de constru\u00e7\u00e3o&quot; que ocorrem em diferentes etapas do desenvolvimento embrion\u00e1rio. 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