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      Síndrome de West: a propósito de nove casos Translated title: West syndrome: report of nine eases

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          Abstract

          A síndrome de West é forma de epilepsia generalizada que se inicia no primeiro ano de vida, com pico de incidência entre 5 e 8 meses, caracterizada por espasmos ou mioclonias maciças, regressão do desenvolvimento neuropsicomotor e alteração eletrencefalográfica denominada hipsarritmia. Relatamos nove casos de síndrome de West, discutindo aspectos clínicos, etiológicos, evolutivos e terapêuticos.

          Translated abstract

          West syndrome is a peculiar form of epilepsy of infancy and childhood characterized by spasms or massive myoclonus, regression neuropsychomotor development, and EEC abnormalities referred as hipsarrhythmia. We report nine cases of West syndrome discussing clinical, etiological, evolutive and therapeutic features.

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          Most cited references19

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          ON A PECULIAR FORM OF INFANTILE CONVULSIONS,

          W.J. West (1841)
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            Precise characterization and quantification of infantile spasms.

            With the use of a time-synchronized video and polygraphic recording system, 5,042 infantile spasms were monitored and analyzed in 24 infants aged 1 to 43 months. Of these, 33.9% were flexor, 22.5% extensor, and 42.0% mixed flexor-extensor. Sometimes the spasms were followed by a period of akinesia and diminished responsiveness lasting up to 90 seconds, and rarely (1.0%) this "arrest" effect constituted the entire seizures. More than one type of seizure occurred in 21 of the 24 infants. In the same number, 78.3% of the seizures occurred in clusters, and the intensity and frequency of the spasms in each cluster often increased to a peak, then progressively decreased until they stopped. Predominantly, the clusters occurred soon after arousal from sleep. The number of seizures occurring at night (55.2%) was similar to the diurnal number (44.8%). The electroencephalographic seizure pattern was variable, but a marked generalized attenuation of electrical activity was a feature of 71.7% of the attacks. Attenuation episodes of similar degree and duration occurred with no evidence of a seizure.
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              A prospective study of infantile spasms: clinical and therapeutic correlations.

              C Lombroso (1983)
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                Author and article information

                Journal
                anp
                Arquivos de Neuro-Psiquiatria
                Arq. Neuro-Psiquiatr.
                Academia Brasileira de Neurologia - ABNEURO (São Paulo, SP, Brazil )
                0004-282X
                1678-4227
                September 1993
                : 51
                : 3
                : 352-357
                Affiliations
                [01] orgnameFaculdade de Medicina de Marília
                Article
                S0004-282X1993000300011 S0004-282X(93)05100311
                a99a3ee9-e053-4b5f-8a88-e02935e2a540

                This work is licensed under a Creative Commons Attribution 4.0 International License.

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                Figures: 0, Tables: 0, Equations: 0, References: 19, Pages: 6
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                SciELO Brazil


                epilepsia,infantile spasms,síndrome de West,espasmos infantis,epilepsy,West syndrome

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