title: Cingulate epileptogenesis in hypothalamic hamartoma.
abstract: Hypothalamic hamartoma (HH) is a relatively rare cause of epilepsy, mainly affecting children. Nearly all patients develop gelastic seizures, often followed by other focal seizure types. Our case illustrates the mechanisms of epileptogenesis in HH. The patient developed gelastic attacks as a baby, and secondarily generalized seizures and drop attacks at 9 years of age. Magnetic resonance imaging (MRI) confirmed the presence of a HH. Presurgical assessment with intracranial electroencephalography (EEG) monitoring recorded gelastic seizures with generalized epileptiform activity. Functional stimulation of the hamartoma provoked gelastic attacks. Single pulse electrical stimulation (SPES) was used to identify epileptogenic cortex. SPES of the left cingular cortex provoked generalized responses similar to the spontaneous generalized discharges. Our results suggest that long-standing history of epilepsy in patients with HH may be related to additional sources of epileptogenic activity. Electrical stimulation performed in this patient provided additional data to favor the hypothesis of secondarily epileptogenesis in the cingulate gyrus independently from the primary origin in the HH.
authors.affiliation: Department of Clinical Neuroscience, Institute of Psychiatry, King's College London, London, United Kingdom.
authors.forename: Antonio
authors.initials: A
authors.lastname: Valentin
authors.forename: Marian
authors.initials: M
authors.lastname: Lazaro
authors.forename: Nandini
authors.initials: N
authors.lastname: Mullatti
authors.forename: Sebastian
authors.initials: S
authors.lastname: Cervantes
authors.forename: Irfan
authors.initials: I
authors.lastname: Malik
authors.forename: Richard P
authors.initials: RP
authors.lastname: Selway
authors.forename: Gonzalo
authors.initials: G
authors.lastname: Alarcón
mesh_terms: D000328:Adult; D001931:Brain Mapping; D046690:Deep Brain Stimulation / Q000379:methods; D004567:Electrodes, Implanted; D004569:Electroencephalography; D017034:Epilepsy, Frontal Lobe / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery; D006179:Gyrus Cinguli / Q000503:physiopathology / Q000601:surgery; D006222:Hamartoma / Q000150:complications* / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D007027:Hypothalamic Diseases / Q000150:complications / Q000503:physiopathology / Q000601:surgery; D007845:Laughter / Q000502:physiology; D008279:Magnetic Resonance Imaging; D008297:Male; D016896:Treatment Outcome