title: Tracking the source of cerebellar epilepsy: hemifacial seizures associated with cerebellar cortical dysplasia.
abstract: Traditionally, subcortical structures such as the cerebellum are supposed to exert a modulatory effect on epileptic seizures, rather than being the primary seizure generator. We report a 14-month old girl presenting, since birth, with seizures symptomatic of a right cerebellar dysplasia, manifested as paroxystic contralateral hemifacial spasm and ipsilateral facial weakness. Multimodal imaging was used to investigate both anatomical landmarks related to the cerebellar lesion and mechanisms underlying seizure generation. Electric source imaging (ESI) supported the hypothesis of a right cerebellar epileptogenic generator in concordance with nuclear imaging findings; subsequently validated by intra-operative intralesional recordings. Diffusion spectrum imaging-related tractography (DSI) showed severe cerebellar structural abnormalities confirmed by histological examination. We suggest that hemispheric cerebellar lesions in cases like this are likely to cause epilepsy via an effect on the facial nuclei through ipsilateral and contralateral aberrant connections.
authors: EEG and Epilepsy Unit, Department of Clinical Neurosciences, University Hospitals and Faculty of Medicine of Geneva, Switzerland.
authors: Agustina M
authors: AM
authors: Lascano
authors: Alia
authors: A
authors: Lemkaddem
authors: Cristina
authors: C
authors: Granziera
authors: Christian M
authors: CM
authors: Korff
authors: Colette
authors: C
authors: Boex
authors: Benoit
authors: B
authors: Jenny
authors: Thomas
authors: T
authors: Schmitt-Mechelke
authors: Jean-Philippe
authors: JP
authors: Thiran
authors: Valentina
authors: V
authors: Garibotto
authors: Maria Isabel
authors: MI
authors: Vargas
authors: Karl
authors: K
authors: Schaller
authors: Margitta
authors: M
authors: Seeck
authors: Serge
authors: S
authors: Vulliemoz
mesh_terms: D000293:Adolescent; D002525:Cerebellar Cortex / Q000002:abnormalities* / Q000473:pathology*; D002526:Cerebellar Diseases / Q000150:complications / Q000175:diagnosis*; D004827:Epilepsy / Q000175:diagnosis* / Q000209:etiology; D005260:Female; D019569:Hemifacial Spasm / Q000175:diagnosis* / Q000209:etiology; D006801:Humans