Juvenile Huntington's disease confirmed by genetic examination in twins Doença de Huntington juvenil confirmada por exame genético em gêmeas

Early-onset Huntington's disease (HD) occurs in approximately 10% of HD's cases. We report juvenile HD in phenotypically identical twins, evaluated by history, clinical and neurologic examination, mini-mental state examination, blood laboratory exams, cerebrospinal fluid examination, skull...

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Main Authors: GILBERTO LEVY, MARIA EDUARDA NOBRE, VINICIUS T. CIMINI, SALMO RASKIN, ELIASZ ENGELHARDT
Format: Article
Language:English
Published: Thieme Revinter Publicações 1999-09-01
Series:Arquivos de Neuro-Psiquiatria
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Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X1999000500022
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author GILBERTO LEVY
MARIA EDUARDA NOBRE
VINICIUS T. CIMINI
SALMO RASKIN
ELIASZ ENGELHARDT
author_facet GILBERTO LEVY
MARIA EDUARDA NOBRE
VINICIUS T. CIMINI
SALMO RASKIN
ELIASZ ENGELHARDT
author_sort GILBERTO LEVY
collection DOAJ
description Early-onset Huntington's disease (HD) occurs in approximately 10% of HD's cases. We report juvenile HD in phenotypically identical twins, evaluated by history, clinical and neurologic examination, mini-mental state examination, blood laboratory exams, cerebrospinal fluid examination, skull computed tomography, and genetic examination for HD. Patients had the akinetic-rigid variety (Westphal variant) of the disease and paternal inheritance. The laboratory workup confirmed the clinical diagnosis of HD, which adds this report to the rare cases of HD in twins reported in the literature.<br>Doença de Huntington (DH) de início precoce ocorre em aproximadamente 10% dos casos de DH. Relatamos DH juvenil em gêmeas fenotipicamente idênticas, avaliadas por história, exames clínico e neurológico, mini-exame do estado mental, exames de sangue, exame do líquido cefalorraquidiano, tomografia computadorizada de crânio e exame genético para DH. As pacientes apresentavam a variedade rígido-acinética (variante de Westphal) da doença e herança paterna. A avaliação laboratorial confirmou o diagnóstico clínico de DH, acrescentando-se este relato aos raros casos de DH em gêmeos relatados na literatura.
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spelling doaj-art-0fd317326ca5419981893f09b6e35cf32025-08-20T01:58:48ZengThieme Revinter PublicaçõesArquivos de Neuro-Psiquiatria0004-282X1678-42271999-09-01573B86786910.1590/S0004-282X1999000500022Juvenile Huntington's disease confirmed by genetic examination in twins Doença de Huntington juvenil confirmada por exame genético em gêmeasGILBERTO LEVYMARIA EDUARDA NOBREVINICIUS T. CIMINISALMO RASKINELIASZ ENGELHARDTEarly-onset Huntington's disease (HD) occurs in approximately 10% of HD's cases. We report juvenile HD in phenotypically identical twins, evaluated by history, clinical and neurologic examination, mini-mental state examination, blood laboratory exams, cerebrospinal fluid examination, skull computed tomography, and genetic examination for HD. Patients had the akinetic-rigid variety (Westphal variant) of the disease and paternal inheritance. The laboratory workup confirmed the clinical diagnosis of HD, which adds this report to the rare cases of HD in twins reported in the literature.<br>Doença de Huntington (DH) de início precoce ocorre em aproximadamente 10% dos casos de DH. Relatamos DH juvenil em gêmeas fenotipicamente idênticas, avaliadas por história, exames clínico e neurológico, mini-exame do estado mental, exames de sangue, exame do líquido cefalorraquidiano, tomografia computadorizada de crânio e exame genético para DH. As pacientes apresentavam a variedade rígido-acinética (variante de Westphal) da doença e herança paterna. A avaliação laboratorial confirmou o diagnóstico clínico de DH, acrescentando-se este relato aos raros casos de DH em gêmeos relatados na literatura.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X1999000500022doença de HuntingtonjuvenilgêmeosHuntington's diseasejuveniletwins
spellingShingle GILBERTO LEVY
MARIA EDUARDA NOBRE
VINICIUS T. CIMINI
SALMO RASKIN
ELIASZ ENGELHARDT
Juvenile Huntington's disease confirmed by genetic examination in twins Doença de Huntington juvenil confirmada por exame genético em gêmeas
Arquivos de Neuro-Psiquiatria
doença de Huntington
juvenil
gêmeos
Huntington's disease
juvenile
twins
title Juvenile Huntington's disease confirmed by genetic examination in twins Doença de Huntington juvenil confirmada por exame genético em gêmeas
title_full Juvenile Huntington's disease confirmed by genetic examination in twins Doença de Huntington juvenil confirmada por exame genético em gêmeas
title_fullStr Juvenile Huntington's disease confirmed by genetic examination in twins Doença de Huntington juvenil confirmada por exame genético em gêmeas
title_full_unstemmed Juvenile Huntington's disease confirmed by genetic examination in twins Doença de Huntington juvenil confirmada por exame genético em gêmeas
title_short Juvenile Huntington's disease confirmed by genetic examination in twins Doença de Huntington juvenil confirmada por exame genético em gêmeas
title_sort juvenile huntington s disease confirmed by genetic examination in twins doenca de huntington juvenil confirmada por exame genetico em gemeas
topic doença de Huntington
juvenil
gêmeos
Huntington's disease
juvenile
twins
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X1999000500022
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