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Epileptic and imaging findings in perinatal hypoxic-ischemic encephalopathy with ulegyria
Flavio Villani1, Ludovico D'Incerti, Tiziana Granata
1Istituto Nazionale Neurologico, Via Celoria 11, Milan, Italy. fvillani@istituto-besta.it
Insights
Hypoxic-ischemic encephalopathy (HIE) in full-term infants can lead to ulegyria, a condition characterized by severe, drug-refractory epilepsy. The extent of ulegyric lesions correlates with epilepsy severity in these infants.
Area of Science:
- Neurology
- Neuroscience
- Pediatrics
Background:
- Hypoxic-ischemic encephalopathy (HIE) is a significant cause of neonatal mortality and long-term disability.
- Ulegyria, a neuropathological feature of HIE, typically affects full-term infants and involves specific patterns of brain damage.
Purpose of the Study:
- To characterize the electroclinical features of HIE when ulegyria is a primary neuropathological finding.
- To investigate the relationship between ulegyric lesions and epilepsy in affected infants.
Main Methods:
- Retrospective analysis of nine patients with MRI-proven ulegyria and epilepsy.
- Comprehensive neurological evaluation, including assessment of clinical features and lesion extent.
Main Results:
- Ulegyric lesions were predominantly found in parasagittal watershed areas, often co-occurring with other HIE-related injuries.
- Patients commonly presented with mental retardation, motor deficits, and partial epilepsy refractory to medication.
- Epilepsy severity directly correlated with the extent of ulegyria and associated HIE lesions.
Conclusions:
- Ulegyria in HIE is associated with a complex clinical presentation, notably severe, drug-refractory epilepsy.
- The degree of brain damage, specifically ulegyria, is a key determinant of epilepsy severity in this population.
Abstract:
Hypoxic-ischemic encephalopathy due to fetal or neonatal asphyxia is a major cause of acute mortality and chronic disability involving cerebral palsy, seizures, and mental retardation. The gestational age of the infant is one of the main variables determining the neuropathological picture of hypoxic-ischemic brain injury, and ulegyria (one of its neuropathological correlates) typically affects full-term infants. The damage usually involves the deeper sulcal portion of the convolutions while sparing the crowns, and includes subcortical white matter atrophy and gliosis. The aim of this study was to characterize the electroclinical features of hypoxic-ischemic encephalopathy when ulegyria is one of its main neuropathological features. To this end, nine patients with MRI-proven ulegyria and epilepsy underwent a complete neurological work-up. The ulegyric lesions were mainly distributed in the parasagittal watershed areas and frequently associated with other hypoxic-ischemic lesions. The neurological picture was characterized in most patients by mental retardation, motor deficits, and drug-refractory partial epilepsy. The ulegyria in our patients was associated with a complex clinical picture: epilepsy was a prominent component, and its severity directly correlated with the extent of the ulegyria and the associated hypoxic-ischemic lesions. Drug refractoriness was an almost constant correlate of this form of symptomatic epilepsy.