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Refractory spasms of focal onset-A potentially curable disease that should lead to rapid surgical evaluation
Mathilde Chipaux1, Georg Dorfmüller2, Martine Fohlen1
1Pediatric Neurosurgery Unit, Rothschild Foundation Hospital, Paris, France.
Insights
Early surgery for infantile spasms (IS) with focal onset seizures leads to favorable outcomes. Prompt surgical intervention, ideally within 36 months of the first seizure, significantly improves seizure control in children.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neurosurgery
Background:
- Infantile spasms (IS) can be the sole seizure type in children with surgically treatable epilepsies.
- While early surgical intervention shows promise, outcomes remain under-researched.
Purpose of the Study:
- To investigate outcomes in children with infantile spasms (IS) of focal onset undergoing epilepsy surgery.
- To identify factors influencing postsurgical seizure control in this pediatric population.
Main Methods:
- Retrospective review of children with IS and focal onset seizures treated between 2002 and 2014.
- Analysis of patient demographics, lesion characteristics, surgical details, and seizure outcomes (Engel classification).
Main Results:
- 68 children with focal onset IS were analyzed; 86% underwent surgery.
- Favorable seizure outcome (Engel 1a) was achieved in 74.6% of patients, rising to 87.9% with surgery within 36 months.
- Hemispheric lesions were associated with earlier age of onset and younger surgical age.
Conclusions:
- Surgery is a viable and effective option for selected pediatric patients with focal onset infantile spasms.
- Postsurgical outcomes for focal onset IS are comparable to other seizure types.
- Timely surgical intervention is crucial for optimizing seizure control.
Purpose:
Infantile spasms (IS) can occur as the only seizure type in children with surgically amenable epilepsies. Although early surgery has shown positive effects, little is known regarding outcomes.
Methods:
We retrospectively reviewed all children with IS referred to our tertiary center between 2002 and 2014 and try to define factors of outcome.
Results:
Sixty-eight children with focal onset seizures were referred: twenty children with a hemispheric implication and 48 with one or more lobes involved. The age of onset was significantly earlier in the hemispheric population (8.0 versus 16.7 months in the focal population). There was no difference in the age of onset between anterior and posterior onset zones, as we could expect regarding the maturation gradient. The epilepsy began earlier in life in tuberous sclerosis than in DNET. Only three children of the 48 non-hemispheric patients had a normal MRI at the time of the surgery. Temporal lobe was involved only in a third of the population. More than 86% of the patients were operated on. Patients with hemispheric lesions were operated on younger (2.6 years+/- 2.1 years) compared to 4.6+/- 3.5 years in the whole population. The most frequent etiologies were in descending order: dysplasia, ganglioglioma or dysembryoplastic tumours and tuberous sclerosis. The global seizure outcome was favorable (Engel 1a) in 74.6% of the patients, and 87.9% if the delay between the first seizure and the surgery was less than 36 months. It fell to 64.7% if the delay exceeded 50 months.
Conclusion:
Spasms of focal onset have a similar postsurgical outcome as other seizure types so surgery may be an excellent option for treating selected patients with focal infantile spasms. Volume and type but not topography of the lesion influence the age of onset. MRI is very helpful to locate the pathology in the pediatric population, since only a small portion had a normal MRI.