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Characteristics of epilepsy in focal cortical dysplasia in infancy
Anne Lortie1, Perrine Plouin, Catherine Chiron
1Paediatric Neurology and EEG Services, Hôpital Sainte-Justine, 3175 Côte Sainte-Catherine, Montreal, Que, Canada H3T 1C5. lortie.a@sympatico.ca
Insights
Focal cortical dysplasia (FCD) causes early, difficult-to-treat infantile epilepsy. While infantile spasms (IS) respond to medication, partial seizures often require early surgery for better neurodevelopmental outcomes.
Area of Science:
- Neurology
- Pediatric Epilepsy
- Neurodevelopmental Disorders
Background:
- Focal cortical dysplasia (FCD) is a leading cause of epilepsy in infants.
- Characteristics of FCD-related epilepsy in infancy are not well-documented.
- Understanding FCD epilepsy is crucial for timely intervention and management.
Purpose of the Study:
- To delineate the clinical characteristics of epilepsy associated with FCD in infants.
- To evaluate the response to medical and surgical treatments for FCD-induced seizures.
- To assess the neurodevelopmental outcomes in infants with FCD and epilepsy.
Main Methods:
- Retrospective study of 28 infants diagnosed with FCD.
- Analysis of seizure semiology, age of onset, and psychomotor development.
- Evaluation of treatment responses (medical and surgical) and neurodevelopmental findings.
Main Results:
- All patients presented with early partial seizures; semiology varied with dysplasia location.
- Infantile spasms (IS) occurred in 11 patients, were mainly asymmetrical, and responded to Vigabatrin or ACTH.
- Partial seizures were refractory to medical treatment; 15/28 underwent surgery, with 8 becoming seizure-free and 7 showing psychomotor improvement.
Conclusions:
- Early-onset, refractory partial seizures characterize FCD in infancy, with easily controlled IS.
- Focal ictal semiology can aid in localizing FCD; intrinsic epileptogenicity likely drives the clinical pattern.
- Given poor medical control and neurodevelopmental prognosis, early surgical consideration is vital for infants with FCD.
Abstract:
To describe the poorly known characteristics of epilepsy during infancy in focal cortical dysplasia (FCD), one of the most frequent cause of infantile epilepsy. All 28 patients with FCD referred to two specialized centres were retrospectively studied regarding seizure characteristics, psychomotor evaluation, and response to medical and surgical treatment. All patients presented with early partial seizures. Semiology, but not the age of onset, depended on the topography of the dysplasia, with abnormal eye movements in all cases of posterior FCD. Eleven patients also developed infantile spasms (IS), mainly asymmetrical. IS were easily controlled with Vigabatrin or ACTH, but no partial seizures could be medically controlled except in one patient. All patients except one had abnormal neuropsychological findings. Fifteen patients had surgery, eight became seizure free, and seven were significantly improved regarding psychomotor development. Very early and refractory partial seizures, but easily controlled IS are the main characteristics of FCD in infancy. Only the focal ictal semiology may help differentiate the localization of FCD. Its intrinsic epileptogenicity could sustain this clinical pattern. Since the chances for medical control and normal neurodevelopment are poor, surgical treatment should be considered early in infants with FCD.
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