Related Experiment Videos
Children with familial cryptogenic epilepsy have a favorable seizure prognosis
D S MacIntosh1, P R Camfield, C S Camfield
1Department of Pediatrics, Dalhousie University Medical School, IWK Grace Health Centre, Halifax, Nova Scotia, Canada.
Insights
Children with epilepsy and a family history, but no clear genetic cause, show a very good prognosis for seizure remission. Most children and their relatives achieved long-term seizure freedom.
Area of Science:
- Neurology
- Genetics
- Pediatrics
Background:
- Epilepsy in children without a defined genetic syndrome poses diagnostic and prognostic challenges.
- Family history of epilepsy is a known risk factor, but its impact on prognosis in idiopathic cases is less understood.
Purpose of the Study:
- To investigate the long-term seizure remission prognosis for children with epilepsy who have a first-degree relative with epilepsy, but no identified genetic syndrome.
- To compare the prognosis of these probands with their affected relatives and with similar patients from a historical cohort.
Main Methods:
- A population-based cohort of 504 children with epilepsy in Nova Scotia was studied, identifying probands (n=27) with epilepsy and an affected first-degree relative (n=32).
- Seizure remission was assessed after an average follow-up of 15 years for probands and 26 years for relatives.
- Outcomes were compared between probands, relatives, and a historical cohort using statistical analysis.
Main Results:
- A high rate of seizure remission was observed: 92% of probands and 76% of affected relatives were seizure-free for 3+ years.
- Early seizure onset (before age 12) was associated with even higher remission rates: 96% for probands and 94% for relatives.
- Remission rates in this group were significantly better than for similar patients in the original Nova Scotian cohort (P < .02).
Conclusions:
- Children with epilepsy, lacking a defined genetic syndrome but having a first-degree relative with epilepsy, demonstrate a favorable long-term prognosis.
- The presence of an affected family member, in the absence of a known genetic cause, is not indicative of a poor outcome.
- This finding suggests that familial aggregation of epilepsy, even without a specific syndrome, may be associated with a better seizure control trajectory.
Abstract:
We examined the prognosis for children with epilepsy not conforming to a genetic syndrome, who also had close relatives with epilepsy. Probands, with no identified cause for epilepsy except a first-degree relative with epilepsy, were identified from a population-based cohort of 504 children in Nova Scotia, with onset of seizures between 1977 and 1985. The primary outcome measure was seizure remission after an average of 15 years follow-up for probands (n = 27) and 26 years for their affected relatives (n = 32). Of probands, 92% were seizure free for 3 or more years at the end of follow-up, compared with 76% of relatives. When seizures began before age 12 years, 96% of probands and 94% of affected relatives were seizure free at the end of follow-up. There was little concordance for the details of the clinical course between probands and affected family members. This high level of remission was considerably better than for similar patients from the original Nova Scotian cohort (P < .02). We conclude that children with epilepsy not conforming to a well-defined genetic syndrome, but with an affected first-degree relative, have a remarkably good prognosis.