• JoomlaWorks Simple Image Rotator
  • JoomlaWorks Simple Image Rotator
  • JoomlaWorks Simple Image Rotator
  • JoomlaWorks Simple Image Rotator
  • JoomlaWorks Simple Image Rotator
  • JoomlaWorks Simple Image Rotator
  • JoomlaWorks Simple Image Rotator
  • JoomlaWorks Simple Image Rotator
  • JoomlaWorks Simple Image Rotator
  • JoomlaWorks Simple Image Rotator
 
  Bookmark and Share
 
 
Master's Dissertation
DOI
10.11606/D.17.2018.tde-10102018-153711
Document
Author
Full name
Larissa Cristina de Queiroga Mendonça Coutinho
E-mail
Institute/School/College
Knowledge Area
Date of Defense
Published
Ribeirão Preto, 2018
Supervisor
Committee
Hamad, Ana Paula Andrade (President)
Leite, João Pereira
Pinho, Ricardo Silva
Saggioro, Fabiano Pinto
Title in Portuguese
Associação entre hemimegalencefalia e esclerose tuberosa em pacientes com epilepsia
Keywords in Portuguese
Epilepsia
Esclerose tuberosa
Hemimegalencefalia
Abstract in Portuguese
Introdução: A hemimegalencefalia é uma rara malformação cerebral que envolve o crescimento anormalmente maior de um hemisfério cerebral. Clinicamente, observase macrocefalia, retardo mental, hemiparesia contralateral, hemianopsia e epilepsia intratável. A Esclerose tuberosa é uma doença genética rara, multissistêmica, caracterizada por tumores benignos e manifesta-se clinicamente com epilepsia e atraso cognitivo. A associação das duas patologias é rara, requer acompanhamento multidisciplinar e tratamentos medicamentoso e cirúrgico específicos. Objetivos: Identificar a presença de hemimegalencefalia em pacientes portadores de esclerose tuberosa com epilepsia atendidos no Centro de Cirurgia de Epilepsia (CIREP) do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo. Realizar revisão de literatura sobre esclerose tuberosa e hemimegalencefalia, assim como a associação entre elas. Material e Métodos: Foi realizada revisão da literatura sobre esclerose tuberosa e hemimegalencefalia e também dos prontuários de pacientes que apresentam associação entre essas patologias. Resultados: Foram analisadas 1534 monitorizações por videoeletroencefalograma (VEEG) em crianças e adolescentes no CIREP. Dessas, 25 apresentavam hemimegalencefalia, e apenas duas com esclerose tuberosa associada. Foram, então, relatados os casos com descrição das etapas de avaliações pré-operatórias, cirurgia para o tratamento da epilepsia, e poslterior seguimento clínico, e também dos exames de imagem, neurofisiológicos e neuropatológicos. Conclusões: A associação entre hemimegalencefalia e esclerose tuberosa é uma manifestação rara, com apenas seis casos descritos na literatura. Contudo, tratandose de associação entre as doenças, a abordagem terapêutica e o seguimento dessas crianças com hemimegalencefalia se modifica, tanto na investigação quanto no acompanhamento clínico das manifestações sistêmicas da esclerose tuberosa. Os dois casos descritos apresentaram espasmos assimétricos, sendo iniciado tratamento com vigabatrina e posterior indicação cirúrgica.
Title in English
Association between hemimegalencephaly and tuberous sclerosis in patients with epilepsy
Keywords in English
Epilepsy
Hemimegalencephaly
Tuberous sclerosis
Abstract in English
Introduction: Hemimegalencephaly is a rare brain malformation that involves an abnormally larger growth of one cerebral hemisphere. Clinically, macrocephaly, mental retardation, contralateral hemiparesis, hemianopsia and intractable epilepsy are observed. Tuberous sclerosis is a rare, multisystemic genetic disease characterized by benign tumors and clinically manifested with epilepsy and cognitive delay. The association of these two pathologies is rare, requiring multidisciplinary care and specific drug and surgical treatment. Objectives: To identify the presence of hemimegalencephaly in patients with tuberous sclerosis with epilepsy attended at the Center for Epilepsy Surgery (CIREP, acronym in Portuguese) of the Clinics Hospital of Ribeirão Preto Medical School. To perform a literature review on tuberous sclerosis and hemimegalencephaly, as well as the association between them. Material and Methods: Review of the literature on tuberous sclerosis and hemimegalencephaly; review of patient records that show the association between these pathologies. Results: 1534 monitoring by VEEG in children and adolescents in CIREP were analyzed. Of these, 25 had hemimegalencephaly, and only two had associated tuberous sclerosis. The cases were reported with descriptions of the stages of preoperative evaluations, surgery for the treatment of epilepsy, with subsequent clinical care, as well as image, neurophysiological and neuropathological examinations. Conclusions: The association between hemimegalencephaly and tuberous sclerosis is a rare manifestation, with only six cases described in the literature. However, considering that it is an association between diseases, the therapeutic approach and the follow-up of these hemimegalencephalic children are specified, both in the investigation and in the clinical care of the systemic manifestations of tuberous sclerosis. Both described cases presented asymmetrical spasms, initiated treatment with vigabatrin with subsequent surgical indication and both presented renal alterations.
 
WARNING - Viewing this document is conditioned on your acceptance of the following terms of use:
This document is only for private use for research and teaching activities. Reproduction for commercial use is forbidden. This rights cover the whole data about this document as well as its contents. Any uses or copies of this document in whole or in part must include the author's name.
Publishing Date
2018-12-21
 
WARNING: Learn what derived works are clicking here.
All rights of the thesis/dissertation are from the authors
CeTI-SC/STI
Digital Library of Theses and Dissertations of USP. Copyright © 2001-2023. All rights reserved.