[Central nervous system malformations: neurosurgery correlates]. / Malformaciones del sistema nervioso central: correlación neuroquirúrgica.

Congenital malformations of the central nervous system are related to alterations in neural tube formation, including most of the neurosurgical management entities, dysraphism and craniosynostosis; alterations of neuronal proliferation; megalencefaly and microcephaly; abnormal neuronal migration, lissencephaly, pachygyria, schizencephaly, agenesis of the corpus callosum, heterotopia and cortical dysplasia, spinal malformations and spinal dysraphism. We expose the classification of different central nervous system malformations that can be corrected by surgery in the shortest possible time and involving genesis mechanisms of these injuries getting better studied from neurogenic and neuroembryological fields, this involves connecting innovative knowledge areas where alteration mechanisms in dorsal induction (neural tube) and ventral induction (telencephalization) with the current way of correction, as well as the anomalies of cell proliferation and differentiation of neuronal migration and finally the complex malformations affecting the posterior fossa and current possibilities of correcting them.

TITLE: Malformaciones del sistema nervioso central: correlacion neuroquirurgica.Las malformaciones congenitas del sistema nervioso central se relacionan con alteraciones en la formacion del tubo neural, en las que se incluyen la mayoria de las entidades de tratamiento neuroquirurgico, disrafismos y craneosinostosis, alteraciones de la proliferacion neuronal, microcefalias y megalencefalias, anomalias de la migracion neuronal, lisencefalia, paquigiria, esquisencefalia, agenesia del cuerpo calloso, heterotopias y displasias corticales, malformaciones raquimedulares y disrafias medulares. En el presente trabajo se clasifican las diferentes malformaciones del sistema nervioso central susceptibles de corregirse mediante cirugia en el menor tiempo posible y se exponen los mecanismos de genesis de estas lesiones cada vez mejor estudiadas desde las areas neurogenetica y neuroembriologica. Esto involucra la posible conexion de areas de conocimiento novedosas, como los mecanismos de alteracion de la induccion dorsal (cierre del tubo neural) y ventral (telencefalizacion) con los mecanismos actuales de correccion, y tambien las anomalias de la proliferacion y diferenciacion celular de la migracion neuronal y, finalmente, el complejo de malformaciones que afectan la fosa posterior y las posibilidades actuales de correccion de estas.

Congenital bilateral perisylvian syndrome: familial occurrence, clinical and psycholinguistic aspects correlated with MRI.

OBJECTIVE: Congenital bilateral perisylvian syndrome (CBPS) is frequently caused by polymicrogyria (PMG). The aim of this study was to correlate the clinical and psycholinguistic aspects with neuroradiological data of patients with CBPS. METHODS: Thirty-one patients were studied. We performed a clinical investigation of the patients and their families, including MRI scanning, neuropsychological tests and language evaluation. RESULTS: The statistical analysis showed that: a) prenatal events are associated with the non-familial type of PMG; b) diffuse PMG is associated with pseudobulbar signs, as opposed to BPPP; c) motor deficit is associated with diffuse PMG; d) epilepsy is equally present in patients with both familial or non-familial PMG, but is more frequently seen in patients with diffuse PMG; e) dyslexia and SLI can be a feature of both the diffuse or BPPP, and either familial or sporadic cases of PMG. CONCLUSIONS: The severity of clinical manifestations in CBPS is correlated with the extent of cortical involvement. Most patients with CBPS have a history of speech delay or language difficulties and no epilepsy. Dyslexia can be found in patients with PMG.

Terminology and classification of the cortical dysplasias.

BACKGROUND: There have been difficulties in achieving a uniform terminology in the literature regarding issues of classification with respect to focal cortical dysplasias (FCDs) associated with epilepsy. OBJECTIVE: S: To review and refine the current terminology and classification issues of potential clinical relevance to epileptologists, neuroradiologists, and neuropathologists dealing with FCD. METHODS: A panel discussion of epileptologists, neuropathologists, and neuroradiologists with special expertise in FCD was held. RESULTS: The panel proposed 1) a specific terminology for the different types of abnormal cells encountered in the cerebral cortex of patients with FCD; 2) a reappraisal of the different histopathologic abnormalities usually subsumed under the term "microdysgenesis," and suggested that this terminology be abandoned; and 3) a more detailed yet straightforward classification of the various histopathologic features that usually are included under the heterogeneous term of "focal cortical dysplasia." CONCLUSION: The panel hopes that these proposals will stimulate the debate toward more specific clinical, imaging, histopathologic, and prognostic correlations in patients with FCD associated with epilepsy.

Ultrasonografía transfontanelar en las malformaciones del desarrollo del SNC en neonatos / Transfontanelar ultrasound in CNS malformations

Propósito. Demostrar el valor de la Ultrasonografía (US) en el diagnóstico precoz de las malformaciones del desarrollo del sistema nervioso central y determinar la incidencia de las mismas en la población estudiada. Material y métodos. Desde abril de 1999 a abril de 2003 fueron realizadas por el mismo operador 5726 ecografías cerebrales de primera vez con transductores microconvex de 5 y 6 MHz por vía trans-fontanelar. Presentamos un análisis retrospectivo de las mismas referidas solamente a la detección de malformaciones del desarrollo cerebral en niños de 0 solicitadas por diferentes motivos. Se excluyeron las alteraciones cerebrales secundarias a patologías que no inciden en el desarrollo del SNC. (Hipoxia, encefalopatía, atrofia, etc.). Resultados. Se detectaron 118 malformaciones estructurales del desarrollo cerebral (2 por ciento). Siendo su frecuencia la siguiente: Hidrocefalia, 40 casos (34 por ciento); Agenesia parcial o total de cuerpo calloso, 19 (16 por ciento); Hidranencefalia, 13 (11 por ciento); Encefalocele, 12 (10 por ciento); Holoprosencefalia, 11 (9,3 por ciento); Complejo Dandy Walker, 10 (8,5 por ciento); Agenesia de Septum, 5 (4,2 por ciento); Esquisencefalia, 3 (2,5 por ciento); Lipomas intracraneanos, 2 (1,6 por ciento); Malformación Vascular de la fosa posterior, 1 (0,8 por ciento); Quiste aracnoideo, 1 (0,8 por ciento) y la asociación de Dandy Walker con Holoprosencefalia en un paciente, 1 (0,8 por ciento)...(AU)