Made available in DSpace on 2015-07-22T20:49:30Z (GMT). No. of bitstreams: 0
Previous issue date: 2010-06-30. Added 1 bitstream(s) on 2015-08-11T03:26:22Z : No. of bitstreams: 1
Publico-238a.pdf: 2039350 bytes, checksum: 86bdc74274c3689283e5ff87f9e33f79 (MD5). Added 1 bitstream(s) on 2015-08-11T03:26:22Z : No. of bitstreams: 2
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Publico-238a.pdf: 2039350 bytes, checksum: 86bdc74274c3689283e5ff87f9e33f79 (MD5)
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Publico-238s.pdf: 1240894 bytes, checksum: 934b73572d5b717f099e2a652d713d89 (MD5) / Objetivo: Investigar se variações fenotípicas, incluindo expressão de traços reflexos têm implicações prognósticas na epilepsia mioclônica juvenil (EMJ). Métodos: Setenta e seis pacientes com EMJ participaram de um protocolo neuropsicológico por vídeo-EEG (VNPP) e métodos habituais de ativação. Sessenta e cinco deles foram acompanhados por pelo menos três anos. Os traços reflexos foram definidos como indução de crises e/ou descargas ao EEG pelo fechamento ocular, fotoestimulação intermitente, linguagem, praxia ou cálculo. Os pacientes tiveram avaliação psiquiátrica e responderam ao IDATE (Inventário Diagnóstico de Ansiedade Traço-Estado). O controle de crises foi classificado de acordo com Prasad e cols. (2003). Os pacientes foram divididos em Grupo 1: controle satisfatório de crises e Grupo 2: controle moderado ou pobre de crises. A avaliação por Video-EEG/EEG foi repetida após uma média de 4,4 anos em 21 pacientes. Resultados: Vinte e nove dos 76 (38,2%) pacientes apresentaram efeito provocativo e a inibição foi encontrada em 28/31 (90,3 %). Os métodos habituais de ativação foram mais eficazes em provocar descargas no EEG. As tarefas de programação-ação, especialmente aquelas envolvendo a praxia manual foram mais eficazes como métodos de ativação que as de pensamento. O efeito inibitório foi mais freqüente durante o cálculo mental. Os pacientes com distúrbios de ansiedade tiveram maiores índices de descarga e não tiveram efeito inibitório pelo VNPP. Quarenta de 65 (61,5%) pacientes acompanhados atingiram controle satisfatório de crises, 25 (38,5%) deles tornaram-se livres de crises. Os pacientes do Grupo 2 tiveram duração mais longa da epilepsia; maior prevalência da combinação dos três tipos de crises; descargas no EEG de base; registro de crises e sensibilidade à praxia. Comparados aos pacientes livres de crises, aqueles com crises persistentes apresentaram início mais precoce da epilepsia; maior prevalência de transtornos de personalidade; maiores escores no IDATE-Traço e maior prevalência da sensibilidade à praxia e à linguagem. A repetição do EEG/video-EEG sugeriu uma evolução paralela entre o desaparecimento dos traços reflexos e o controle das crises. Conclusões: Dentre as tarefas neuropsicológicas, a praxia exerceu o efeito provocativo mais marcado. A expressão de traços reflexos e algumas características fenotípicas em pacientes com EMJ apresentam implicações negativas para o prognóstico. / Purpose: To investigate if phenotypic variations including reflex traits expression have prognostic implications in Juvenile Myoclonic Epilepsy (JME). Methods: Seventy-six patients with JME underwent a video-EEG neuropsychological protocol (VNPP) and habitual methods of activation. Sixty-five of them were followed for at least three years. Reflex traits were defined as seizures and/or EEG discharges induction by eye-closure, photic stimulation, language, praxis or calculation. Patients had psychiatric evaluation and answered to STAI (State-Trait Anxiety Inventory). Seizure control was classified according to Prasad et al. (2003). Patients were divided into Group 1: good seizure control and Group 2: moderate or poor seizure control. Video-EEG/EEG evaluation was repeated after a mean of 4.4 yrs. in 21 patients. Results:. Twenty-nine out of the 76 (38.2%) presented provocative effect and inhibition was seen in 28/31 (90.3 %). Habitual methods of activation were more effective in provoking discharges than VNPP. Action-programming tasks, especially those involving manual praxis, were more effective as activation method than thinking. Inhibitory effect was higher in mental calculation. Patients with anxiety disorders had greater discharge indexes and no significant inhibitory effect on VNPP. Forty of 65 (61.5%) patients followed reached good seizure control, 25 (38.5%) of whom became seizure free. Group 2 patients had longer epilepsy duration; higher prevalence of the combination of all three seizure types; discharges in baseline EEG; seizure recording and sensitivity to praxis. Compared to seizure-free patients, those with persistent seizures presented younger age at epilepsy onset; higher prevalence of personality disorders; higher scores in STAI-Trait and higher incidence of sensitivity to praxis and to language tasks. Repetition of EEG/video-EEG suggested a parallel evolution of reflex traits disappearance and seizure control. Conclusions: Praxis exerted the most remarkable provocative effect, in accordance with the hypothesis of a motor circuitry hyperexcitability in JME. This study suggests that reflex traits expression and some other phenotypic features in JME patients have negative implications in prognosis. / TEDE / BV UNIFESP: Teses e dissertações
Identifer | oai:union.ndltd.org:IBICT/oai:repositorio.unifesp.br:11600/9018 |
Date | 30 June 2010 |
Creators | Guaranha, Mirian Salvadori Bittar [UNIFESP] |
Contributors | Universidade Federal de São Paulo (UNIFESP), Yacubian, Elza Márcia Targas [UNIFESP] |
Publisher | Universidade Federal de São Paulo (UNIFESP) |
Source Sets | IBICT Brazilian ETDs |
Language | Portuguese |
Detected Language | English |
Type | info:eu-repo/semantics/publishedVersion, info:eu-repo/semantics/doctoralThesis |
Format | 199 p. |
Source | reponame:Repositório Institucional da UNIFESP, instname:Universidade Federal de São Paulo, instacron:UNIFESP |
Rights | info:eu-repo/semantics/openAccess |
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