Please use this identifier to cite or link to this item: http://repositorio.unicamp.br/jspui/handle/REPOSIP/196358
Type: Artigo de periódico
Title: [perisylvian Syndrome: Report Of One Brazilian Family With Focus On The Genetic Mode Of Inheritance And Clinical Spectrum].
Author: Herrera, Eliane P
Brandão-Almeida, Iara L
Guimarães, Catarina A
Oliveira, Ecila P M
Montenegro, Maria Augusta
Cendes, Fernando
Lopes-Cendes, Iscia
Guerreiro, Carlos A M
Hage, Simone R V
Guerreiro, Marilisa M
Abstract: Perisylvian syndrome (PS) refers to a variety of clinical manifestations associated with lesions in the perisylvian or opercular region. Acquired lesions such as cerebrovascular diseases or virus encephalitis and congenital lesions such as polymicrogyria (PMG) may be implied as etiological factors. The onset of the PS may occur in early childhood. The aim of this study was to report one family with PS in order to draw attention to this rarely diagnosed entity. Our family has five affected patients, three children and two male adults. All of them had developmental language disorder. Epilepsy, motor deficit and pseudobulbar signs (such as drooling) were detected in one child who had diffuse PMG along the Sylvian fissure. Subtle clinical manifestations correlated with either subtle MRI findings or normal MRI. Most reported families provide evidence suggestive of X-linked transmission. However, the most likely mode of inheritance in our family is autosomal dominant, since a male to male transmission was documented.
Subject: Adult
Brain Diseases
Brazil
Cerebral Cortex
Child
Epilepsy, Rolandic
Female
Humans
Magnetic Resonance Imaging
Male
Medulla Oblongata
Mouth Diseases
Movement Disorders
Nervous System Malformations
Pedigree
Speech Disorders
Syndrome
Rights: aberto
Identifier DOI: /S0004-282X2005000300017
Address: http://www.ncbi.nlm.nih.gov/pubmed/16059598
Date Issue: 2005
Appears in Collections:Artigos e Materiais de Revistas Científicas - Unicamp

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