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Epilepsy surgery in infants
E Wyllie1, Y G Comair, P Kotagal
1Department of Neurology, Cleveland Clinic Foundation, OH 44195, USA.
Insights
Epilepsy surgery in infants can effectively control severe seizures, with many achieving seizure freedom or significant improvement. This intervention offers hope for developmental progress in carefully selected young patients.
Area of Science:
- Pediatric Neurosurgery
- Epileptology
- Developmental Neurology
Background:
- Infantile epilepsy, particularly catastrophic forms, presents significant challenges.
- Early diagnosis and intervention are crucial for optimal outcomes in affected infants.
Purpose of the Study:
- To evaluate the efficacy and safety of epilepsy surgery in infants with catastrophic epilepsy.
- To assess surgical outcomes in relation to underlying etiologies and seizure characteristics.
Main Methods:
- Surgical resection or functional hemispherectomy was performed on 12 infants (age 2.5-29 months).
- Preoperative evaluation included MRI, PET, and EEG to localize epileptogenic lesions.
- Histopathologic examination confirmed diagnoses such as focal cortical dysplasia, Sturge-Weber syndrome, ganglioglioma, and hemimegalencephaly.
Main Results:
- At a mean follow-up of 32 months, 6 patients were seizure-free (Engel class I), 3 had rare seizures (Engel class II), and 2 showed worthwhile improvement (Engel class III).
- Most patients experienced developmental catch-up post-surgery, except for one with hemimegalencephaly.
- Complications were rare and manageable; no new neurological deficits occurred, and one patient's hemiparesis resolved.
Conclusions:
- Epilepsy surgery is a viable treatment option for catastrophic epilepsy in carefully selected infants.
- Surgical intervention can lead to seizure control and improved developmental trajectories.
Purpose:
We report 12 infants who had frontal (n = 3), temporal (n = 2), or temporoparieto-occipital (n = 2) resection or functional hemispherectomies (n = 5) at age 2.5-29 (mean 15.3) months for catastrophic epilepsy due to focal cortical dysplasia (n = 5), Sturge-Weber syndrome (n = 3), ganglioglioma (n = 3), or hemimegalencephaly (n = 1). Seizures began at 1 day to 14 months (mean, 4.0 months) after birth, occurred frequently (often many times per day, and were refractory to antiepileptic drugs. Patients were evaluated for surgery at 2.5-24 (mean 12.4) months of age. Seven patients had hemiparesis and eight had slowed cognitive development. Seizures were characterized by arrest or marked reduction of behavioral motor activity with nuclear level of consciousness (n = 4, with temporal or temporoparietal EEG seizures), focal clonic activity (n = 3, with perirolandic EEG seizures), generalized tonic stiffening (n = 3, with temporoparieto-occipital, parietal, or frontal EEG seizures), or infantile spasms and hypsarrhythmia (n = 2, with a frontal tumor or temporoparieto-occipital cortical dysplasia).
Methods:
Magnetic resonance imaging (MRI) revealed the epileptogenic lesion in all but two patients, both with cortical dysplasia localized by interictal positron-emission tomography (PET) and other clinical or EEG features and confirmed on histopathologic examination of resected tissue.
Results:
At follow-up 4-86 (mean 32) months after surgery, six patients were seizure free (Engel outcome class I), three had rare seizures with none in at least the previous 6 months (Engel class II), and two had worthwhile improvement (Engel class III). Except for the severely developmentally delayed infant with hemimegalencephaly, several patients had marked "catch-up" developmental progress after class I, II, or III outcome. Postoperative complications included subdural hematoma over the contralateral hemisphere (one patient) and entrapment and enlargement of the ipsilateral temporal horn (one patient) after hemispherectomy, both corrected uneventfully with a second surgical procedure. One patient died of unexplained causes several hours after frontal lobectomy. No patients had new neurologic deficits after surgery, and one patient had resolution of progressive fluctuating hemiparesis after resection of temporoparieto-occipital cortical dysplasia.
Conclusions:
Our results agree with previous reports that epilepsy surgery can provide relief from catastrophic epilepsy in carefully selected infants.