Related Experiment Videos
Congenital porencephaly and hippocampal sclerosis. Clinical features and epileptic spectrum
S S Ho1, R I Kuzniecky, F Gilliam
1Department of Neurology, University of Alabama at Birmingham, USA.
Insights
Porencephaly patients with intractable seizures often have coexisting mesial temporal sclerosis, identified as the seizure focus. This finding suggests surgical options for these complex epilepsy cases.
Area of Science:
- Neurology
- Neuroimaging
- Epilepsy Research
Background:
- Porencephaly is a rare brain malformation associated with intractable seizures.
- Identifying the precise seizure focus is crucial for effective treatment in epilepsy patients.
Purpose of the Study:
- To investigate clinical features and seizure localization in patients with porencephaly and intractable epilepsy.
- To explore the relationship between porencephaly, mesial temporal sclerosis, and seizure characteristics.
Main Methods:
- Studied 14 patients with porencephaly and intractable seizures.
- Utilized electroencephalography (EEG) and magnetic resonance imaging (MRI) for seizure localization and brain volumetry.
- Assessed clinical features, including neurological deficits and seizure types.
Main Results:
- Complex partial seizures (CPS) were the most common type (10 patients).
- EEG indicated temporal onset in nine patients; MRI revealed porencephaly in various cerebral artery distributions.
- Hippocampal and amygdalar atrophy were frequent, often correlating with CPS semiology and temporal EEG findings.
Conclusions:
- Mesial temporal sclerosis frequently coexists with porencephaly and is often the seizure focus in concordant cases.
- Recognition of dual pathology enables surgical intervention for select patients with porencephaly-related epilepsy.
- Perinatal cerebral vascular occlusion may underlie the common ischemic pathogenesis of porencephaly and mesial temporal sclerosis.
Abstract:
We studied clinical features and seizure localization in 14 patients with porencephaly and intractable seizures. Perinatal complications were present in nine patients, childhood febrile convulsions in two, congenital hemiparesis in 12, and intellectual impairment in seven. Ten patients had psychoparetic complex partial seizures (CPS), three had sensorimotor simple partial seizures, and one had generalized tonic-clonic seizures. Surface EEG showed temporal onset in nine patients (one bitemporal) and extratemporal onset in four. MRI showed porencephaly in the distribution of the middle cerebral artery in eight patients, posterior cerebral in three, internal carotid in one, and multiple vessels in two. MR-based volumetry revealed hippocampal formation atrophy in 13 patients (eight unilateral and five bilateral) and amygdalar atrophy in 10 patients (nine unilateral and one bilateral). Hippocampal formation atrophy was concordant with CPS semiology in 10 patients (71%) and with EEG temporal localization in nine patients. Two patients had pathologic confirmation of mesial temporal sclerosis and were seizure free after temporal lobectomy. We conclude that mesial temporal sclerosis often coexists with porencephaly and is the likely seizure focus in the presence of concordant electroclinical data. This recognition implies that effective surgical intervention can be offered to certain patients with porencephaly-related seizure disorders. The dual pathology and association with perinatal cerebral vascular occlusion suggest a common ischemic pathogenesis.