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Epidemiologic features of infantile spasms in Slovenia
Zvonka Rener Primec1, Stefan Kopac, David Neubauer
1Department of Child Neurology, University Children's Hospital, Ljubljana, Slovenia. zvonka.rener@mf.uni-lj.si
Insights
This study analyzed infantile spasms (ISs) in Slovenia, finding an incidence of 2.06 per 10,000 live births. Outcomes for infantile spasms significantly depend on the underlying cause, with symptomatic cases showing poorer prognoses.
Area of Science:
- Pediatric Neurology
- Epidemiology
- Clinical Pediatrics
Background:
- Infantile spasms (ISs) are a severe epilepsy syndrome in infants.
- Understanding the epidemiology and outcomes of ISs is crucial for public health and clinical management.
Purpose of the Study:
- To determine the epidemiologic features of infantile spasms (ISs) in Slovenia.
- To assess the outcomes of children diagnosed with ISs in Slovenia.
Main Methods:
- Retrospective study of medical records for all children with ISs in Slovenia (1985-1995).
- Inclusion of data from all pediatric EEG laboratories and community pediatrician referrals.
- Follow-up assessment conducted in 1998 to evaluate outcomes.
Main Results:
- Identified 47 children with ISs over 11 years; cumulative incidence was 2.06 per 10,000 live births.
- Symptomatic etiology (61.7%) included tuberous sclerosis, vascular insults, cerebral malformations, and Down syndrome.
- Of 47 children, 38.3% were seizure-free at follow-up, with outcomes strongly linked to etiology; 4 children died.
Conclusions:
- The incidence of ISs in Slovenia is comparable to some US regions but lower than in Nordic countries.
- Etiology is the primary determinant of outcome in infantile spasms.
- Further neuroimaging is recommended for cryptogenic cases to elucidate etiology.
Purpose:
To determine the epidemiologic features of infantile spasms (ISs) in Slovenia.
Methods:
Medical records of all children with ISs in Slovenia in the period from 1985 to 1995, based on community pediatrician referrals to four hospitals, including all pediatric EEG laboratories, were retrospectively studied. The outcome was assessed by a follow-up study in 1998.
Results:
Forty-seven children with ISs were identified over an 11-year interval. The cumulative incidence was 2.06 per 10,000 live births. Among 29 (61.7%) children with symptomatic etiology, 14 cases had prenatal etiology [tuberous sclerosis (TS), seven; vascular insult, three; cerebral malformations, three; Down syndrome, one child], 14 perinatal and one postnatal cause: anoxic brain damage after cardiac surgery. Cryptogenic and idiopathic etiology were diagnosed in 13 (27.6%) and five (10.6%) of 47 cases, respectively. The age of onset of ISs ranged from 2 to 10 months. As initial treatment, steroids were used in 19 children (remission in 10); vigabatrin in seven (remission in four), and other antiepileptic drugs (AEDs) in 20 children (remission in six). According to the follow-up study, 18 (38.3%) children were seizure free, and 14 of them had normal mental development. Among 29 mentally retarded children (14 severely), 26 belonged to the symptomatic group. Four children died.
Conclusions:
The incidence of ISs in Slovenia is similar to that in some parts of the United States, but lower than that in Finland or Sweden. The outcome depends mainly on etiology. Additional neuroimaging studies are needed for evaluation of cryptogenic cases.