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Epilepsy and perisylvian lipoma/cortical dysplasia complex
M Guye1, J L Gastaut, F Bartolomei
1Centre Saint-Paul, Centre Hospitalier Spécialisé dans l'Epilepsie, Marseille, France.
Summary
Hemispheric cerebral lipomas, rare congenital malformations, are frequently associated with epilepsy. Non-surgical management is recommended due to surgical risks and the benign nature of these intracranial tumors.
Area of Science:
- Neurology
- Neuroimaging
- Congenital Malformations
Background:
- Intracranial lipomas are rare congenital malformations composed of mature adipocytes.
- While typically midline, hemispheric locations are infrequent (3-7%), with only 21 prior reported cases.
- Epilepsy is a common clinical manifestation of hemispheric cerebral lipomas.
Observation:
- Two patients presented with new-onset partial motor seizures attributed to sylvian region hemispheric lipomas.
- Magnetic resonance imaging (MRI) revealed lipid-signal lesions within the sylvian fissure and adjacent cortical abnormalities (pachygyria-like).
- One patient experienced seizure worsening and hemiparesis after partial lesion excision.
Findings:
- Hemispheric lipomas, though rare, show a high association with epilepsy.
- MRI is crucial for diagnosing these lipid-rich lesions and identifying associated cortical dysplasia.
- Surgical intervention carries significant risks, including seizure exacerbation and neurological deficits.
Implications:
- Non-surgical management is favored for hemispheric lipomas due to benign nature, diagnostic certainty, surgical complexity, and risks of poor outcomes.
- Epilepsy mechanisms likely involve associated vascular and cortical dysplasias, suggesting classification with localized cortical malformations.
- Further research into the pathogenesis and optimal management of these rare lesions is warranted.