West syndrome associated with Down syndrome: Case report and literature review

Hamouda, H. Ben; Mnasri, Hanen; Ghanmi, S.; Soua, H.; Sakli, Gazi; Dogui, Mohamed; Sfar, Mohamed Tahar

doi:10.1016/j.pepo.2014.03.006

Cited by 2 publications

(5 citation statements)

References 21 publications

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“…It was unsuccessful in three cases when used without prednisone 10,53 . In one instance, it did successfully abort epileptic spasms, but relapse was experienced 16 months later and required valproate and hydrocortisone for control 8 …”

Section: Resultsmentioning

confidence: 99%

“…The majority of children with DS and epileptic spasms exhibit hypsarrhythmia on EEG, but modified hypsarrhythmia is also prevalent 4,,8,10–13,15,18,24,25,48–56 . The presence of either hypsarrhythmia or modified hypsarrhythmia, however, is not prognostically valuable.…”

Section: Resultsmentioning

confidence: 99%

“…There have been many case reports and smaller reviews of the literature published about epileptic spasms in individuals with Down syndrome, but there are no recent reviews that compile the available data to gain insight about the lifetime prevalence, current standard of care, and outcomes of individuals with this condition 8–11 . This comprehensive review of the available literature aims for several objectives: to provide an updated understanding of the pathogenesis of epileptic spasms; to describe the clinical characteristics and diagnosis of the disorder in this population; and to discuss the treatment response and developmental outcomes in patients with DS and epileptic spasms.…”

Section: Introductionmentioning

confidence: 99%

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Epileptic spasms in individuals with Down syndrome: A review of the current literature

2020

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Epilepsy can occur in individuals with Down syndrome (DS), with epileptic spasms representing the most frequent seizure type in this population. Epileptic spasms can have devastating consequences on the development of individuals with the condition. This review sought to explore the lifetime prevalence and underlying mechanism of epileptic spasms in this population. We also aimed to review the response rate to various treatments, the relapse rate, and the development of subsequent epilepsy or autism in this population. A comprehensive literature search was conducted for articles discussing the lifetime prevalence, diagnosis, treatment, outcomes, or underlying etiology of epileptic spasms in animal models or individuals with DS. According to available literature, the global clinic‐based lifetime prevalence of epilepsy in individuals with DS ranged from 1.6% to 23.1%, with epileptic spasms representing 6.7%‐66.7% of these cases. Response rate to treatment with adrenocorticotropic hormone/corticosteroids was highest (81%) and has the most literature supporting its use, with other regimens, including vigabatrin and other antiepileptic drugs, having lower response rates. Epileptic spasms occur more frequently in children with DS than in the general population, though more studies are needed to determine the true lifetime prevalence of epileptic spasms in this population. Generally, children with DS and epileptic spasms tend to be more responsive to treatment and have better outcomes than children with epileptic spasms of unknown etiology (ie, without DS), in terms of response and relapse rates as well as the development of intractable epilepsy (eg, Lennox‐Gastaut syndrome).

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Epileptic spasms in individuals with Down syndrome: A review of the current literature

2020

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“…The prevalence of epilepsy in patients with Down syndrome (DS) is low (4.1%) but of these, 44% usually represent with infantile spasms, and the long-term prognosis is poor, even with early diagnosis and early treatment. [2][3][4] The rare situation in which two monozygotic twins were diagnosed with DS and WS could become more frequent in the next few years because there is an increase in the average age of pregnant women. Moreover, the initial response to phenobarbital in the sister, led to its use in the other patient, which also showed effectiveness and was sustained for the 2 years of follow up.…”

mentioning

confidence: 99%

“…Phenobarbital treatment is poorly described in the literature as effective in stopping crises in WS. Hamouda et al 4 described a case report in which a patient with Down syndrome associated with WS responded temporarily, for 16 months, with complete resolution of the spasms; however, after this period there was a recurrence of the crises. The patient (Twin 2) in this report has been in complete remission of the case for more than 2 years.…”

mentioning

confidence: 99%