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West syndrome and other infantile epileptic encephalopathies--Indian hospital experience
V Kalra1, S Gulati, R M Pandey
1Child Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi 110029, India. vkalra@medinst.ernet.in
Insights
Infantile epileptic encephalopathies, especially West syndrome, often stem from prenatal or perinatal issues. Early diagnosis and treatment are crucial for better seizure control and developmental outcomes in affected children.
Area of Science:
- Pediatric Neurology
- Epileptology
- Developmental Neuroscience
Background:
- Infantile epileptic encephalopathies (IEEs) represent a significant portion of pediatric neurology consultations.
- West syndrome is the most common IEE, frequently symptomatic with prenatal or perinatal causes.
Purpose of the Study:
- To analyze seizure semiology, etiological factors, and treatment outcomes in children with IEEs.
- To compare the efficacy of different therapeutic modalities on spasm frequency, development, and seizure status.
- To identify factors influencing final seizure outcome and long-term development.
Main Methods:
- Retrospective analysis of case records for 94 children diagnosed with IEEs.
- Evaluation of seizure semiology, prenatal/perinatal insults, developmental status, and investigations.
- Comparison of therapeutic interventions including ACTH, prednisolone, conventional antiepileptics, and vigabatrin.
Main Results:
- West syndrome accounted for 55.3% of IEEs, with two-thirds being symptomatic.
- Prenatal factors (66.6%) were more common than perinatal factors (33.3%) in etiology.
- ACTH and prednisolone showed better initial spasm control (54.5% and 52.9%) than conventional antiepileptics (25.3%).
- Delayed development at onset (OR=4), delayed diagnosis (>12 months) (OR=2.27), and Lennox-Gastaut syndrome (OR=4.75) were associated with poor final seizure outcomes.
- Combined ACTH/prednisolone and antiepileptic treatment showed better final seizure response (36.6%) than antiepileptics alone (20%).
- Initial psychomotor retardation (OR=23.4) and abnormal EEG (OR=7.46) predicted delayed development on follow-up.
Conclusions:
- Early diagnosis and intervention are critical for improving seizure control and developmental outcomes in infantile epileptic encephalopathies.
- While corticosteroids offer initial spasm control, their long-term impact on development and final seizure status is limited.
- Identifying neurometabolic etiologies is important for targeted therapy, and delays in diagnosis significantly worsen prognosis.
Abstract:
Children with infantile epileptic encephalopathies comprising 3.5% of the Pediatric Neurology Clinic registrations in a tertiary care hospital were retrospectively analyzed. Data were retrieved from case records and analyzed for seizure semiology, prenatal and perinatal insults, developmental status and relevant investigations. The various therapeutic modalities and their influence on spasm frequency, long-term development and final seizure status were compared. The two primary outcome variables analyzed included final seizure status and developmental outcome. Of the 94 infantile epileptic encephalopathies, West syndrome was the commonest (55.3%), of which two thirds were symptomatic. Etiological factors were prenatal in 66.6% and perinatal in 33.3%. The initial response to ACTH was good in 54.5% with subsequent relapse in 27.8% and for prednisolone was 52.9 and 44.4%, respectively, compared to 25.3% spasms control with conventional antiepileptic drugs. Disease category of infantile epileptic encephalopathies evolved in 4, i.e. early myoclonic encephalopathy to West syndrome 1, early infantile epileptic encephalopathy to West syndrome 1, West syndrome to Lennox-Gastaut syndrome 2. Psychomotor retardation was seen in 88.2%, with 16.1% having normal development at onset of spasms. Microcephaly was associated with delayed development but did not influence final seizure outcome. Final seizure outcome was poor in children with delayed development at onset (adjusted odds ratio [OR]=4), delay in diagnosis >12 months (OR=2.27) and in children with Lennox-Gastaut syndrome (OR=4.75). ACTH/prednisolone and antiepileptic treatment versus antiepileptics alone showed a good final seizure response in 36.6% versus 20%. Development on follow up was delayed in children with initial psychomotor retardation (OR=23.4) and abnormal electroencephalogram (OR=7.46). Perinatal factors constituted one third of symptomatic West syndrome. The use of ACTH/corticosteroids resulted in good initial spasm control though final seizure outcome and development were unaffected. Prednisolone had similar response to ACTH in spasm control but higher subsequent relapse rate. Vigabatrin was useful though often unaffordable. The identification of a neurometabolic etiology, though uncommon, has significant therapy implications. Delay in diagnosis was common and negatively influenced final seizure outcome.