Infantile spasms in Down syndrome—effects of delayed anticonvulsive treatment

Eisermann, Monika; DeLaRaillère, A.; Dellatolas, Georges; Tozzi, E.; Nabbout, Rima; Dulac, Olivier; Chiron, Catherine

doi:10.1016/s0920-1211(03)00088-3

Cited by 121 publications

(112 citation statements)

References 43 publications

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“…Among prognostic factors, the treatment lag and duration of spasms have been recognized as being significant (Lombroso, 1983;Eisermann et al, 2003;Goh et al, 2005). In most studies a treatment lag of up to one month correlates with a better prognosis.…”

Section: Discussionmentioning

confidence: 99%

“…Prognosis largely depends on etiology, being most unfavorable in prenatal cases with evolution to resistant epilepsy and severe mental retardation. However, infants with cryptogenic/idiopathic etiology can have favorable outcome with lifelong remission of seizures and normal or nearly normal mental development (Lombroso, 1983;Dulac et al, 1993;Riikonen, 1996).Among prognostic factors, the treatment lag and duration of spasms have been recognized as being significant (Lombroso, 1983;Eisermann et al, 2003;Goh et al, 2005). In most studies a treatment lag of up to one month correlates with a better prognosis.…”

mentioning

confidence: 99%

“…In most studies a treatment lag of up to one month correlates with a better prognosis. Prolonged duration of spasms despite treatment increases the risk of mental retardation (Eisermann et al, 2003;Goh et al, 2005). In some infants hypsarrhythmia continues despite the cessation of spasms.…”

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confidence: 99%

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The Risk of Lower Mental Outcome in Infantile Spasms Increases after Three Weeks of Hypsarrhythmia Duration

2006

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Summary:To assess the correlation between hypsarrhythmia duration and mental outcome in infantile spasms (IS) the medical records of 48 infants with IS were reviewed retrospectively and psychological assessments undertaken at follow-up at the age of 3 to 13 years. We found 18 (38%) cryptogenic IS cases with typical hypsarrhythmia and 30 symptomatic with modified hypsarrhythmia-further classified into 15 cases as multifocal, 10 as pseudoperiodic and 5 as unilateral hypsarrhythmia. A short treatment lag (one to two weeks) occurred in 25, three to four weeks in 10 cases. Spasms ceased within one month after treatment in 23 infants. At follow-up 15 children had normal mental outcome (borderline included). A correlation between hypsarrhythmia duration longer than three weeks and lower mental outcome was found using the logistic regression model. The duration of hypsarrhythmia represents a sensitive prognostic parameter in IS; the risk of mental retardation increases after three weeks of hypsarrhythmia. Key Words: Infantile spasmsDuration of hypsarrhythmia-Mental outcome-Children.Infantile spasms (IS), i.e., West syndrome, as an epileptic encephalopathy, represents one of the major causes of acquired mental retardation in early childhood (Roger and Dulac, 1994). Prognosis largely depends on etiology, being most unfavorable in prenatal cases with evolution to resistant epilepsy and severe mental retardation. However, infants with cryptogenic/idiopathic etiology can have favorable outcome with lifelong remission of seizures and normal or nearly normal mental development (Lombroso, 1983;Dulac et al., 1993;Riikonen, 1996).Among prognostic factors, the treatment lag and duration of spasms have been recognized as being significant (Lombroso, 1983;Eisermann et al., 2003;Goh et al., 2005). In most studies a treatment lag of up to one month correlates with a better prognosis. Prolonged duration of spasms despite treatment increases the risk of mental retardation (Eisermann et al., 2003;Goh et al., 2005). In some infants hypsarrhythmia continues despite the cessation of spasms. Therefore we explored the impact of hypsarrhythmia duration on mental outcome in 48 children with IS; the epidemiologic part of the study was published in 2002 (Rener Primec et al., 2002 MATERIALS AND METHODSThe medical records of 48 infants diagnosed with IS were studied retrospectively. IS were defined as a syndromic condition (Roger and Dulac, 1994;Lux and Osborne, 2004). The methodology applied has been described previously (Rener Primec et al., 2002), when epidemiologic features of 47 children (permanent residency in Slovenia) were presented. One additional child (immigrant) was included here. The essentials for this study were standard 12-channel EEG recordings taken in wakefulness and sleep at the time of diagnosis of IS and subsequent EEG recordings obtained every 7 to 10 days during the first 2 months or until remission (whichever came first), then monthly for the first 6 months after diagnosis of IS. If the spasms reoccurred an infant was admi...

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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The Risk of Lower Mental Outcome in Infantile Spasms Increases after Three Weeks of Hypsarrhythmia Duration

2006

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“…Another limitation is that the induced spasms are observed in 2-month-old mice (late adolescent to adult). Epileptic spasms in humans can be observed after infancy [62,63] but have not been reported to occur this early in Down syndrome [64].…”

Section: Models Of Genetic Causes Of Ismentioning

confidence: 97%

Novel Animal Models of Pediatric Epilepsy

et al. 2012

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When mimicking epileptic processes in a laboratory setting, it is important to understand the differences between experimental models of seizures and epilepsy. Because human epilepsy is defined by the appearance of multiple spontaneous recurrent seizures, the induction of a single acute seizure without recurrence does not constitute an adequate epilepsy model. Animal models of epilepsy might be useful for various tasks. They allow for the investigation of pathophysiological mechanisms of the disease, the evaluation, or the development of new antiepileptic treatments, and the study of the consequences of recurrent seizures and neurological and psychiatric comorbidities. Although clinical relevance is always an issue, the development of models of pediatric epilepsies is particularly challenging due to the existence of several key differences in the dynamics of human and rodent brain maturation. Another important consideration in modeling pediatric epilepsy is that "children are not little adults," and therefore a mere application of models of adult epilepsies to the immature specimens is irrelevant. Herein, we review the models of pediatric epilepsy. First, we illustrate the differences between models of pediatric epilepsy and models of the adulthood consequences of a precipitating insult in early life. Next, we focus on new animal models of specific forms of epilepsies that occur in the developing brain. We conclude by emphasizing the deficiencies in the existing animal models and the need for several new models.

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“…[6][7][8][9][10][11] The prevalence of IS ranges from 2 to 3.5 per 10,000 live births per year and is often accompanied by neurodevelopmental regression and hypsarrhythmia on EEG recording. [12][13][14] Successful early treatment can protect ongoing development and lead to permanent remission.…”

Section: Infantile Spasmsmentioning

confidence: 99%