Epilepsy and ring chromosome 20: case report

We present the clinical, electroencephalographic, neuroimaging (brain magnetic resonance image - MRI and spectroscopy by MRI) and cytogenetic findings of a young male patient with a rare cytogenetic anomaly characterised by a de novo 46,XY,r(20)(p13q13.3) karyotype. He presents with mental retardati...

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Main Authors: Gomes Marleide da Mota, Lucca Irene, Bezerra Sonia Alonso Monteiro, Llerena Jr Juan, Moreira Denise Madeira
Format: Article
Language:English
Published: Thieme Revinter Publicações 2002-01-01
Series:Arquivos de Neuro-Psiquiatria
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Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2002000400022
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author Gomes Marleide da Mota
Lucca Irene
Bezerra Sonia Alonso Monteiro
Llerena Jr Juan
Moreira Denise Madeira
author_facet Gomes Marleide da Mota
Lucca Irene
Bezerra Sonia Alonso Monteiro
Llerena Jr Juan
Moreira Denise Madeira
author_sort Gomes Marleide da Mota
collection DOAJ
description We present the clinical, electroencephalographic, neuroimaging (brain magnetic resonance image - MRI and spectroscopy by MRI) and cytogenetic findings of a young male patient with a rare cytogenetic anomaly characterised by a de novo 46,XY,r(20)(p13q13.3) karyotype. He presents with mental retardation, emotional liability, and strabismus, without any other significant dysmorphies. There are brain anomalies characterised by corpus callosum, uvula, nodule and cerebellum pyramid hypoplasias, besides arachnoid cysts in the occipital region. He had seizures refractory to pharmacotherapy and long period of confusional status with or without a motor component. The authors recognised that the EEG pattern was not fixed but changed over time, specially for bursts of slow waves with great amplitude accompanied or not by sharp components, and bursts of theta waves sharply contoured. Previously, epilepsy solely has been assigned to region 20q13. However, the important structural cerebral alterations present in our case has not been reported associated to such chromosomal abnormality and may indicate possible new chromosomal sites where such atypical neurological characteristics could be mapped.
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series Arquivos de Neuro-Psiquiatria
spelling doaj-art-010232ee13f04a42a376ea17844b95a12025-08-20T03:22:31ZengThieme Revinter PublicaçõesArquivos de Neuro-Psiquiatria0004-282X1678-42272002-01-01603A631635Epilepsy and ring chromosome 20: case reportGomes Marleide da MotaLucca IreneBezerra Sonia Alonso MonteiroLlerena Jr JuanMoreira Denise MadeiraWe present the clinical, electroencephalographic, neuroimaging (brain magnetic resonance image - MRI and spectroscopy by MRI) and cytogenetic findings of a young male patient with a rare cytogenetic anomaly characterised by a de novo 46,XY,r(20)(p13q13.3) karyotype. He presents with mental retardation, emotional liability, and strabismus, without any other significant dysmorphies. There are brain anomalies characterised by corpus callosum, uvula, nodule and cerebellum pyramid hypoplasias, besides arachnoid cysts in the occipital region. He had seizures refractory to pharmacotherapy and long period of confusional status with or without a motor component. The authors recognised that the EEG pattern was not fixed but changed over time, specially for bursts of slow waves with great amplitude accompanied or not by sharp components, and bursts of theta waves sharply contoured. Previously, epilepsy solely has been assigned to region 20q13. However, the important structural cerebral alterations present in our case has not been reported associated to such chromosomal abnormality and may indicate possible new chromosomal sites where such atypical neurological characteristics could be mapped.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2002000400022ring chromosome 20epilepsymental retardation
spellingShingle Gomes Marleide da Mota
Lucca Irene
Bezerra Sonia Alonso Monteiro
Llerena Jr Juan
Moreira Denise Madeira
Epilepsy and ring chromosome 20: case report
Arquivos de Neuro-Psiquiatria
ring chromosome 20
epilepsy
mental retardation
title Epilepsy and ring chromosome 20: case report
title_full Epilepsy and ring chromosome 20: case report
title_fullStr Epilepsy and ring chromosome 20: case report
title_full_unstemmed Epilepsy and ring chromosome 20: case report
title_short Epilepsy and ring chromosome 20: case report
title_sort epilepsy and ring chromosome 20 case report
topic ring chromosome 20
epilepsy
mental retardation
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2002000400022
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AT bezerrasoniaalonsomonteiro epilepsyandringchromosome20casereport
AT llerenajrjuan epilepsyandringchromosome20casereport
AT moreiradenisemadeira epilepsyandringchromosome20casereport