Dataset Information

Response to treatment and outcomes of infantile spasms in Down syndrome.

ABSTRACT:

Aim

To estimate the prevalence, and evaluate presentation, treatment response, treatment side effects, and long-term seizure outcomes in all known cases of children with Down syndrome and infantile spasms on the island of Ireland.

Method

This was a 10-year retrospective multicentre review of clinical records and investigations, focusing on treatment response, side effects, and long-term outcomes.

Results

The prevalence of infantile spasms in Down syndrome was 3.0% during the study period. Fifty-four infants were identified with median age of spasm onset at 201 days (interquartile range [IQR] 156-242). Spasm cessation was achieved in 88% (n=46) at a median of 110 days (IQR 5-66). The most common first-line medications were prednisolone (n=20, 37%), vigabatrin (n=18, 33.3%), and sodium valproate (n=9, 16.7%). At follow-up (median age 23.7mo; IQR 13.4-40.6), 25% had ongoing seizures and 85% had developmental concerns. Treatment within 60 days did not correlate with spasm cessation. Seventeen children (31%) experienced medication side effects, with vigabatrin accounting for 52%.

Interpretation

Prednisolone is an effective and well-tolerated medication for treating infantile spasms in Down syndrome. Despite the high percentage of spasm cessation, developmental concerns and ongoing seizures were common.

SUBMITTER: Harvey S

PROVIDER: S-EPMC9303415 | biostudies-literature | 2022 Jun

REPOSITORIES: biostudies-literature

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Publications

Response to treatment and outcomes of infantile spasms in Down syndrome.

Harvey Susan S Allen Nicholas M NM King Mary D MD Lynch Bryan B Lynch Sally A SA O'Regan Mary M O'Rourke Declan D Shahwan Amre A Webb David D Gorman Kathleen M KM

Developmental medicine and child neurology 20220129 6

<h4>Aim</h4>To estimate the prevalence, and evaluate presentation, treatment response, treatment side effects, and long-term seizure outcomes in all known cases of children with Down syndrome and infantile spasms on the island of Ireland.<h4>Method</h4>This was a 10-year retrospective multicentre review of clinical records and investigations, focusing on treatment response, side effects, and long-term outcomes.<h4>Results</h4>The prevalence of infantile spasms in Down syndrome was 3.0% during th ...[more]

PMID: 35092693

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Project description:BackgroundInfantile epileptic spasms syndrome (IESS) is a common epileptic syndrome in infancy. Current first-line treatments include adrenocorticotropic hormone (ACTH), corticosteroids and vigabatrin, with early control of epileptic spasms potentially benefiting long-term outcomes, such as improved psychomotor development. Early treatment, which means the prompt use of first-line treatments, is crucial for achieving an initial response in IESS. However, to date, no clear definition of the specific timeframe that constitutes early treatment has been identified. The objective of this study is to perform a secondary analysis of our previously published IESS cohort data to determine a suitable lead time.MethodsAn analysis was conducted using a cohort of 263 children with IESS who had previously received ACTH first-line treatment. This study investigated whether intervening within a certain treatment time window could potentially increase or decrease the likelihood of a short-term response.ResultsOut of the 263 children with IESS, 108 achieved a short-term response. The lead time of the response group was significantly shorter than that of the non-response group [1.50 (interquartile range, 1.00, 3.00) vs. 2.00 (interquartile range, 1.00, 5.00) months; P=0.003]. A restricted cubic spline graph with several adjusted variables, including time of first spasm and aetiological classification, showed a significant linear relationship between lead time and short-term response and a non-linear trend (inverted U-shaped curve), with a significant inflection point at 1.6 months. Using 1.5 months as the cutoff and dichotomising lead time, the adjusted logistic regression results showed that in children with a lead time >1.5 months, the likelihood of a short-term response decreased with increasing lead time [odds ratio (OR) =0.59, 95% confidence interval (CI): 0.33-0.92, P=0.041), whereas children with a lead time ≤1.5 months showed no significant association between lead time and short-term response (OR =1.03, 95% CI: 0.72-1.47, P=0.89).ConclusionsFor children with IESS, initiating first-line treatment within 1.5 months of the onset of spasms is recommended. For those who start first-line treatment after more than 1.5 months from the onset, the likelihood of a short-term response may significantly decrease as the lead time increases.

Project description:IntroductionChildren with infantile epileptic spasms syndrome (IESS) are likely to experience poor outcomes. Researchers have investigated the factors related to its long-term prognosis; however, none of them developed a predictive model.ObjectiveThis study aimed to clarify the factors that influence the long-term prognosis of seizures and their development and to create a prediction model for IESS.Materials and methodsWe conducted a retrospective cohort study enrolling participants diagnosed with IESS at the Tottori University Hospital. We examined the seizure and developmental status at 3 and 7 years after the IESS onset and divided the participants into favorable and poor outcome groups. Subsequently, we analyzed the factors associated with the poor outcome group and developed a prediction model at 3 years by setting cutoff values using the receiver operating characteristic curve.ResultsData were obtained from 44 patients with IESS (19 female patients and 25 male patients). Three years after epileptic spasms (ES) onset, seizure and development were the poor outcomes in 15 (34.9%) and 27 (61.4%) patients, respectively. The persistence of ES or tonic seizures (TS) after 90 days of onset, moderate or severe magnetic resonance imaging abnormalities, and developmental delay before IESS onset were significantly associated with poor outcomes. Seven years after the onset of ES, seizures and development were the poor outcomes in 9 (45.0%) and 13 (72.2%) patients, respectively. We found that no factor was significantly associated with poor seizure outcomes, and only developmental delay before IESS onset was significantly associated with poor developmental outcomes. Our prediction model demonstrated 86.7% sensitivity and 64.3% specificity for predicting poor seizure outcomes and 88.9% sensitivity and 100% specificity for predicting poor developmental outcomes.ConclusionOur prediction model may be useful for predicting the long-term prognosis of seizures and their development after 3 years. Understanding the long-term prognosis during the initial treatment may facilitate the selection of appropriate treatment.

Dataset Information

Response to treatment and outcomes of infantile spasms in Down syndrome.

Aim

Method

Results

Interpretation

Publications

Response to treatment and outcomes of infantile spasms in Down syndrome.

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