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Published on: September 20, 2024
Favourable prognostic factors with infantile spasms
1Kuopio University Hospital, Kuopio, P.O. Box 1627, FI-70211 Kuopio, Finland. raili.riikonen@kuh.fi
Insights
Favorable outcomes in infantile spasms depend on early treatment and response, regardless of cause. Prognosis can be predicted early, with factors like age at onset and seizure type influencing outcomes.
Area of Science:
- Pediatric Neurology
- Developmental Neuroscience
- Clinical Pediatrics
Background:
- Infantile spasms (IS) are a severe epilepsy syndrome in infants.
- Understanding factors influencing IS prognosis is crucial for management.
Purpose of the Study:
- To review the influence of etiology and treatment on infantile spasms outcomes.
- To identify predictors of outcome and potential strategies for improving prognosis.
Main Methods:
- Review of existing literature on infantile spasms etiology, treatment, and outcome.
- Analysis of factors associated with favorable and unfavorable prognoses.
Main Results:
- Favorable factors include cryptogenic etiology, later onset, absence of atypical seizures, and early treatment response.
- Hormonal therapy may show better short-term outcomes than vigabatrin, though seizure freedom rates are similar.
- Early treatment response predicts cognitive outcome in cryptogenic IS.
Conclusions:
- Prognosis for infantile spasms can be predicted early in the disease course.
- Early intervention and tailored treatment are key to improving outcomes.
- Further research into visual field assessment for vigabatrin risks is needed.
Abstract:
The following aspects are reviewed: Does the aetiology influence the outcome of infantile spasms? Does the treatment influence the outcome? Can the outcome be predicted? Can we improve the prognosis? Favourable factors are the following: cryptogenic aetiology, age at onset > or =4 months, absence of atypical spasms and partial seizures, and absence of asymmetrical EEG abnormalities, short treatment lag, and an early and sustained response to treatment. Not only patients with a cryptogenic aetiology have a favourable outcome. We can already at the first clinical evaluation tell the parents if the prognosis looks favourable. The final goal of the treatment is improved mental outcome. Steroids and vigabatrin are the first-line drugs for infantile spasms in Europe. In a prospective study from the United Kingdom short-term outcome was better with hormonal than with vigabatrin therapy (tuberous sclerosis excluded). However, the numbers of patients who were seizure-free at 3-4 months in different studies have been very similar. Moreover, an early response to treatment seems to be of predictive value for the cognitive outcome in children with cryptogenic spasms. The long-term outcome is known only after hormonal therapy. The side effects of steroids are usually treatable and reversible. In Finland ACTH therapy is given at the minimum effective dose and for the minimum effective time with minimal side effects. The risks of VGB are irreversible visual field defects. As of yet there is no method to examine the visual fields in patients with infantile spasms. Early treatment of infantile spasms seems to be important. Prevention of infantile spasms with some aetiological groups might be possible.
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