The Idiopathic Form of West Syndrome

Vigevano, Federico; Fusco, Lucia; Cusmai, Raffaella; Claps, Dianela; Ricci, Stefano; Milani, L.

doi:10.1111/j.1528-1157.1993.tb00456.x

Cited by 90 publications

(57 citation statements)

References 9 publications

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“…It is well known that the outcome of ISs depends mainly on etiology (10,12,15,19,22). In our study, significantly better outcome, in terms of both mental development and seizure remission, was observed in children in the cryptogenic and idiopathic groups than in the symptomatic group (eight of 13 cryptogenic; all five idiopathic; six of 29 symptomatic cases), which is more than is generally reported (5,19), but similar to data from Iceland (4).…”

Section: Discussioncontrasting

confidence: 54%

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Epidemiologic Features of Infantile Spasms in Slovenia

2002

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Summary:Purpose: To determine the epidemiologic features of infantile spasms (ISs) in Slovenia.Methods: Medical records of all children with ISs in Slovenia in the period from 1985 to 1995, based on community pediatrician referrals to four hospitals, including all pediatric EEG laboratories, were retrospectively studied. The outcome was assessed by a follow-up study in 1998.Results: Forty-seven children with ISs were identified over an 11-year interval. The cumulative incidence was 2.06 per 10,000 live births. Among 29 (61.7%) children with symptomatic etiology, 14 cases had prenatal etiology [tuberous sclerosis (TS), seven; vascular insult, three; cerebral malformations, three; Down syndrome, one child], 14 perinatal and one postnatal cause: anoxic brain damage after cardiac surgery. Cryptogenic and idiopathic etiology were diagnosed in 13 (27.6%) and five (10

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Section: Discussioncontrasting

confidence: 54%

“…Children with symptomatic etiology were further divided, according to identified cause, into prenatal, perinatal, or postnatal groups (12)(13)(14). At the time of follow-up, the idiopathic subgroup was additionally identified from the cryptogenic cases (15).…”

Section: Data Collectionmentioning

confidence: 99%

Epidemiologic Features of Infantile Spasms in Slovenia

2002

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“…El SW es la encefalopatía epiléptica más frecuente y severa de la infancia, conjuntamente con el Síndrome de Ohtahara y el Síndrome de Lennox Gastaut, constituyen las encefalopatías epilépticas dependientes de la edad, que se caracterizan por un tipo de crisis epilépticas muy frecuentes y a menudo refractarias al tratamiento, EEG con patrones específicos y compromiso del desarrollo (25)(26)(27)(28).…”

Section: Discussionunclassified

Síndrome de West, experiencia con una serie de casos con acceso al tratamiento de primera línea, en Lima

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Mendoza²

2015

Rev Neuropsiquiatr

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RESUMENObjetivos: Describir las características clínicas de una serie de pacientes con Síndrome de West (SW) con acceso a la medicación de primera línea. Material y métodos: Estudio retrospectivo observacional, de niños con SW que fueron atendidos entre 1996 y 2014. Resultados: Se incluyeron 37 casos, con una promedio de inicio de espasmos de 6,4 meses, con predominio del sexo masculino (75,7%), la mayoría procedentes de Lima. La etiología más frecuente fue secundaria (83,8%), como prenatales, malformaciones cerebrales, Esclerosis Tuberosa, Síndrome de Down y causas perinatales. El SW fue controlado en 67,6% de los casos; Vigabatrina o ACTH fueron efectivas en 20/32 (62,5%). Dos pacientes fueron controlados con levetiracetam, uno con topiramato, uno con lamotrigina, y uno con cirugía. El patrón electroencefalográfico de hipsarritmia fue predominante 24/37 (64,9%). La comorbilidad neurológica fue muy frecuente (97.3%) y fue de grado leve sólo en 7/37 (18,9%). Dos pacientes fallecieron. Conclusiones: En esta serie el SW fue de causa secundaria y se controló eficientemente con vigabatrina o ACTH, por tanto, se recomienda incluir estos medicamentos en el petitorio nacional. . PALABRAS CLAVE: Síndrome de West, niños, epilepsia, vigabatrina, ACTH, levetiracetam, Perú. SUMMARY Objectives:To describe the clinical characteristics of a series of patients with West Syndrome (WS) with access to first line medication. Material and Methods: Retrospective observational study of children treated between 1996 and 2014. Results: 37 cases were included, with an average starting age of 6.4 months, predominantly males (75.7%), the majority was from Lima. The most common etiology was secondary (83.8%), as prenatal, brain malformations, Tuberous Sclerosis, Down syndrome and perinatal causes. The WS was controlled in 67.6% of the cases; Vigabatrin and ACTH were effective in 20/32 (62.5%). Two patients were controlled with Levetiracetam, one with Topitamate, one with Lamotrigine and one with surgery. Hypsarrhythmia was the predominant electroencephalographic pattern 23/37 (64.9%). Neurologic comorbidities were very frequent (97.3%) and they were mild only in 7/37 (18.9%). Two patients passed away. Conclusions: In this series, the WS had a secondary cause and was efficiently controlled with Vigabatrin or ACTH, therefore, we recommend their inclusion in the national request.

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“…All infants were classified into symptomatic or cryptogenic cases. At follow-up, the idiopathic subgroup was identified, according to published criteria (Vigevano et al, 1993;Lux and Osborne, 2004).…”

Section: Methodsmentioning

confidence: 99%

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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The Idiopathic Form of West Syndrome

Cited by 90 publications

References 9 publications

Epidemiologic Features of Infantile Spasms in Slovenia

Epidemiologic Features of Infantile Spasms in Slovenia

Síndrome de West, experiencia con una serie de casos con acceso al tratamiento de primera línea, en Lima

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

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