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Long-term prognosis after infantile spasms: a statistical study of prognostic factors in 200 cases
Insights
Infantile spasms prognosis is linked to early development, seizure type, and treatment. Prompt ACTH therapy, especially for cryptogenic cases, is crucial for better long-term outcomes in children.
Area of Science:
- Pediatric Neurology
- Developmental Pediatrics
- Clinical Neurophysiology
Background:
- Infantile spasms (IS) are a severe epilepsy syndrome in infants.
- Long-term neurodevelopmental outcomes and prognostic factors for IS require further investigation.
Purpose of the Study:
- To evaluate the long-term condition of children with infantile spasms after age six.
- To identify prognostic factors influencing seizure control, physical and mental development, and educability.
Main Methods:
- A follow-up study of 200 children diagnosed with infantile spasms.
- Comparison of outcomes with various prognostic factors, including ACTH therapy.
- Assessment of seizure status, physical and mental development, and school attendance at final follow-up.
Main Results:
- Complete recovery was achieved in 9.5% of cases; 44.4% of cryptogenic cases recovered fully.
- Poor prognostic factors for seizures included seizure evolution, ACTH relapse, and pre-spasm convulsions.
- Delayed development, neurological abnormalities, and symptomatic etiology were poor prognostic factors for development and educability.
Conclusions:
- Prompt treatment is essential for infantile spasms, particularly cryptogenic forms.
- Identifying prognostic factors aids in predicting long-term outcomes for affected children.
- ACTH therapy shows variable efficacy, emphasizing the need for timely intervention.
Abstract:
A follow-up study was made on 200 children (115 boys, 85 girls) who had had infantile spasms, in order to compare their present condition over the age of six years with various prognostic factors. 48 of the children (30 males and 18 females) had died, and all the rest were aged six years or older at the time of final follow-up. 139 of the children had received ACTH therapy: at final follow-up, spasms had ceased in 43.5 per cent, and about the same proportion showed normal physical development; 23 per cent had normal mental development and 15.4 per cent were attending ordinary schools. Complete recovery (normal mental and physical development and attending ordinary schools) was achieved in only 19 cases (9.5 per cent). Of the cryptogenic cases, 44.4 per cent had made a full recovery. The poor prognostic factors for continuing seizures were evolution into other types of fits, relapse of seizures after ACTH therapy, seizures concomitant with spasms, and convulsions before the onset of spasms. Poor prognostic factors for physical development were delayed development before the onset of spasms, neurological abnormalities, PEG abnormality, symptomatic aetiology, neonatal convulsions, low birthweight, perinatal asphyxia and being female. Poor prognostic factors for mental development were delayed development before the onset of spasms, neurological abnormalities, PEG abnormality, prenatal and perinatal aetiology, relapse after initial ACTH therapy, laughing attacks, and evolution into other types of fits. Only in the cryptogenic cases was there significant correlation between the delay in treatment and the long-term prognosis for mental development. Poor prognostic factors for educability were very similar to those for mental development. In spite of conflicting views as to the long-term effects of ACTH, prompt treatment seems to be mandatory, at least in cryptogenic cases of infantile spasms.