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West syndrome due to perinatal insults
R Cusmai1, S Ricci, J M Pinard
1Service de Neuropédiatrie, Hopital Saint Vincent de Paul, Unité INSERM U29, Paris, France.
Insights
Infantile spasms from perinatal insults can lead to epilepsy. Localized brain lesions, like porencephalic cysts, often have a better epilepsy outcome than diffuse cerebral atrophy.
Area of Science:
- Neurology
- Pediatric Neurology
- Neuroimaging
Background:
- Symptomatic infantile spasms are often caused by perinatal brain insults.
- Understanding the relationship between specific brain lesions and epilepsy outcomes is crucial for patient management.
Purpose of the Study:
- To analyze the clinical, neuroradiological, and epilepsy outcomes in 32 patients with infantile spasms secondary to perinatal insults.
- To correlate specific types and locations of cerebral lesions with the development and prognosis of epilepsy.
Main Methods:
- Retrospective review of clinical data, neuroradiological findings (MRI), and epilepsy outcomes in 32 patients.
- Classification of cerebral lesions into porencephalic cysts, periventricular leukomalacia, and diffuse cerebral atrophy.
- Analysis of epilepsy development and seizure relapse post-infantile spasms.
Main Results:
- Eight patients (25%) developed epilepsy following West syndrome.
- Localized unilateral porencephalic cysts, especially in the rolandic or parieto-occipital regions, were associated with a favorable epilepsy outcome.
- Extensive porencephalic cysts involving the frontal lobe, diffuse cerebral atrophy, and periventricular leukomalacia showed varied epilepsy outcomes, with some patients experiencing persistent epilepsy.
- Patients with periventricular leukomalacia, particularly premature infants, generally had a better epilepsy prognosis.
Conclusions:
- The type and location of cerebral lesions significantly influence the epilepsy outcome in infants with spasms due to perinatal insults.
- Localized lesions appear to confer a better prognosis compared to diffuse or extensive frontal lobe lesions.
- Further research is warranted to elucidate the precise mechanisms linking specific brain damage patterns to epilepsy development and resolution.
Abstract:
Clinical, neuroradiological data and outcome of epilepsy of 32 patients with symptomatic infantile spasms caused by perinatal insults are reported. Neuroradiological investigations showed focal as well as diffuse cerebral lesions. Fifteen children had a porencephalic lesion, 12 had periventricular leukomalacia and five had diffuse bilateral cerebral atrophy, associated with status marmoratus in one child and bilateral ulegyria in another. Eight patients (25%) had epilepsy following West syndrome. In the group of 15 children with porencephalic cysts, the lesion was always unilateral, but topography and extension varied. Eight children had circumscribed cysts involving the rolandic or parieto-occipital regions, and the outcome of epilepsy was favorable. Seven patients had large lesions involving the frontal lobe, associated with unilateral hemispheric atrophy in four cases. These four children had an unfavorable epilepsy outcome. In the 12 patients with periventricular leukomalacia, all premature, eleven had no seizure relapse after the spasms; one of these children had epilepsy. In the five full-term patients with bilateral and diffuse cerebral lesions, three children had epilepsy at the last observation. In children with leukomalacia and in patients with localized porencephalic lesions the outcome of epilepsy appears to be better than in patients with diffuse cerebral lesions or in children with extensive porencephalic cysts, particularly those involving the frontal lobe.