Cognitive outcome in children with infantile spasms using a standardized treatment protocol. A five-year longitudinal study

Bitton, Jonathan; Sauerwein, Hannelore C.; Connolly, Mary; Weiss, Shelly K.; Donner, Elizabeth; Whiting, Sharon; Mohamed, Ismail; Wirrell, Elaine; Ronen, Gabriel M.; Lortie, Anne; Cpen,

doi:10.1016/j.seizure.2021.04.027

Cited by 11 publications

(8 citation statements)

References 30 publications

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“…Deterioration was greater in the subgroup of patients in whom spasms started before seven months compared to the subgroup with seizures that started after seven months. Some studies have reported similar findings regarding the association between age at seizure onset and outcome [22][23][24], with aetiology being the strongest predictor of outcome [11]. Our analysis showed that patients with abnormalities on MRI had a 19-fold increased chance of having developmental delay at the second evaluation compared to children with normal MRI.…”

Section: Discussionsupporting

confidence: 79%

Section: Discussionmentioning

confidence: 67%

“…Existing literature on the outcome of patients with infantile spasms is retrospective. To-date, there are several prospective studies on infantile spasms that have focused on outcome factors [8,10,11,22]. Infantile spasms remain a severe neurological disorder because of their frequent association with delayed development, with a severe impact on the developing brain.…”

Section: Discussionmentioning

confidence: 99%

“…Our analysis showed that patients with abnormalities on MRI had a 19-fold increased chance of having developmental delay at the second evaluation compared to children with normal MRI. Many studies show that structural cases have poorer developmental outcomes [8,10,11,22,25,26]. Koo and colleagues assessed the outcome of patients in the so-called "cryptogenic and symptomatic" groups separately.…”

Section: Discussionmentioning

confidence: 99%

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Predictors of outcome among 31 children with infantile spasms syndrome

Kvernadze

Tatishvili

Lomidze³

et al. 2022

Epileptic Disorders

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Objective. Infantile spasms syndrome is a severe epileptic encephalopathy. Management of infantile spasms remains challenging because of pharmacoresistant forms and relapsing seizures. A high number of patients with this syndrome have neurodevelopmental delay. The main objective of our study was to determine predictors to measure the neurodevelopmental outcome of patients with infantile spasms. Methods. We prospectively evaluated 31 patients with infantile spasms from 2014 to 2017 at three hospitals in Tbilisi, Georgia. Various demographic data were evaluated at the first visit; video‐EEG, brain MRI and neurodevelopmental evaluation were performed upon admission. A diary to record spasms was provided and completed by all parents/caregivers. Seizures were recorded on video and the phenomenology of infantile spasms was studied. Children were followed for one and two years after the first assessment. Results. Neurodevelopmental deterioration was revealed in 61.1% on the second and 53% on the third evaluation in patients with onset of spasms before seven months of age. The mean score on the ASQ communication domain was low among structural cases. Eleven patients with pre‐existing delay had developmental regression based on the second evaluation (Fisher's exact test: 7.2; df 1; p=0.01). Significance. Our study reveals that age at onset of infantile spasms at less than seven months, pre‐existing developmental delay, low ASQ scores and structural abnormalities on MRI are predictors of poor developmental outcome. Our data suggest that clinicians should inform parents at the first clinical evaluation about prognosis, and intervention should be started as early as possible in order to improve development.

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Section: Discussionsupporting

confidence: 79%

Section: Discussionmentioning

confidence: 67%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Predictors of outcome among 31 children with infantile spasms syndrome

Kvernadze

Tatishvili

Lomidze³

et al. 2022

Epileptic Disorders

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“…In conclusion, we have found that PRED and ACTH treatment for IS have similar long-term outcomes for most health conditions, including for most neurological conditions. Further, our study is one of the few for IS, which considers long-term outcomes including that of non-neurological conditions, while most studies are evaluating immediate treatment response (for example, Grinspan et al) ( 13 ) or evaluating long-term outcomes for only cognitive or epilepsy-related aspects ( 14 , 15 ). As some outcomes appeared to be correlated with specific hospitals, future studies can evaluate whether variability in outcomes may be affected by post-treatment differences in care models.…”

Section: Discussionmentioning

confidence: 99%

Long-Term Health Outcomes of Infantile Spasms Following Prednisolone vs. Adrenocorticotropic Hormone Treatment Characterized Using Phenome-Wide Association Study

et al. 2022

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ObjectiveTo determine differences in long-term health and neurological outcomes following infantile spasms (IS) in patients treated with adrenocorticotropic hormone (ACTH) vs. prednisolone/prednisone (PRED).MethodsA retrospective, case-control study of patients with an International Classification of Diseases, Ninth Revision, Clinical Modifications (ICD-9) diagnosis of IS, identified over a 10-year period from a national administrative database, was conducted. IS patients treated with ACTH or PRED were determined and cohorts established by propensity score matching. Outcomes, defined by hospital discharge ICD codes, were followed for each patient for 5 years. Related ICD codes were analyzed jointly as phenotype codes (phecodes). Analysis of phecodes between cohorts was performed including phenome-wide association analysis.ResultsA total of 5,955 IS patients were identified, and analyses were subsequently performed for 493 propensity score matched patients, each in the ACTH and PRED cohorts. Following Bonferroni correction, no phecode was more common in either cohort (p < 0.001). However, assuming an a priori difference, one phecode, abnormal findings on study of brain or nervous system (a category of abnormal neurodiagnostic tests), was more common in the PRED cohort (p <0.05), and was robust to sensitivity analysis. Variability in outcomes was noted between hospitals.SignificanceWe found that long-term outcomes for IS patients following ACTH or PRED treatment were very similar, including for both neurological and non-neurological outcomes. In the PRED-treated cohort there was a higher incidence of abnormal neurodiagnostic tests, assuming an a priori statistical model. Future studies can evaluate whether variability in outcomes between hospitals may be affected by post-treatment differences in care models.

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