Abstract:
:Brainstem dysgenesis designates all those patients with congenital dysfunction of cranial nerves and muscle tone due to prenatal lesions or malformations of the brainstem. This generic term has the advantage over the eponyms Moebius 'expanded' or 'unrestricted', Robin, Cogan or Carey-Fineman-Ziter syndromes in that it has a less restrictive view and provides a frame work that enables a systematic approach to diagnosis and research of most developmental disorders involving the brainstem. The review of the literature and our experience shows that infants with a predominant rombencephalic involvement are due to brainstem prenatal disruptive vascular accidents, while cases with midbrain and cerebellar involvement and widespread malformative syndromes have most likely an underlying genetic cause. Due to phenotypic heterogeneity associated with brainstem dysgenesis, it is crucial to evaluate each case individually and to establish a specific therapeutic plan. Intervention programs should start soon after diagnosis and directed to improve functions needed for daily life activities. Even though the prognosis of patients with brainstem dysgenesis due to prenatal destructive lesions depends on the magnitude of the vascular territory involved, in most patients with brainstem dysgenesis, the prognosis is better than the initial clinical manifestations would indicate. TITLE:Disgenesia troncoencefalica: mas alla del sindrome de Moebius. :El termino 'disgenesia troncoencefalica' se aplica a los pacientes que presentan afectacion congenita de multiples pares craneales, hipotonia muscular y signos leves de afectacion de la via piramidal. Este termino es ventajoso respecto al uso de eponimos tales como Moebius, Robin, Cogan y Carey-Fineman-Ziter, ya que es menos restrictivo y ofrece un nuevo enfoque para comprender las causas y su patogenia, asi como para mejorar el tratamiento de este grupo de alteraciones del desarrollo que afectan exclusiva o predominantemente al tronco del encefalo. La revision de la bibliografia y nuestra experiencia muestran que la mayoria de los casos con afectacion selectiva del rombencefalo se deben a lesiones disruptivas prenatales, mientras que en los casos con afectacion del mesencefalo y el cerebelo, asi como en los sindromes polimalformativos con afectacion destacada del troncoencefalo, la topografia de las lesiones es mas difusa y menos especifica, y la causa hereditaria, mas probable. Debido a la amplia heterogeneidad fenotipica asociada a la disgenesia troncoencefalica, es esencial realizar una evaluacion individualizada y establecer un plan de tratamiento especifico. Los programas de rehabilitacion deben comenzar poco despues del diagnostico y centrarse en mejorar las habilidades motoras, dotando al paciente de las herramientas necesarias para afrontar las necesidades diarias en funcion de la morbilidad asociada. Aunque el pronostico de la disgenesia troncoencefalica secundaria a lesiones disruptivas depende de la localizacion y la extension del territorio vascular afectado, en general, el pronostico de los pacientes con disgenesia troncoencefalica es mejor de lo que las manifestaciones clinicas iniciales harian suponer.
journal_name
Rev Neuroljournal_title
Revista de neurologiaauthors
Munell F,Tormos MA,Roig-Quilis Msubject
Has Abstractpub_date
2018-04-01 00:00:00pages
241-250issue
7eissn
0210-0010issn
1576-6578pii
rn2017273journal_volume
66pub_type
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journal_title:Revista de neurologia
pub_type: 杂志文章
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更新日期:1999-02-01 00:00:00
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pub_type: 杂志文章
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doi:
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pub_type: 历史文章,杂志文章,评审
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pub_type: 临床试验,杂志文章
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更新日期:1999-08-01 00:00:00
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