title: Epilepsy syndromes associated with hypothalamic hamartomas.
abstract: OBJECTIVE
  Hypothalamic hamartoma (HH) related epilepsy presents with gelastic seizures (GS), other seizure types and cognitive deterioration. Although seizure origin in GS has been well established, non-GS are poorly characterized. Their relationship with the HH and cognitive deterioration remains poorly understood. We analyzed seizure type, spread pattern in non-GS and their relationship with the epileptic syndrome in HH.
  
  
  METHODS
  We documented all current seizure types in six adult patients with HH-epilepsy with video-EEG monitoring, characterized clinical-electrographic features of gelastic and non-gelastic seizures and correlated these findings with cognitive profile, as well as MRI and ictal SPECT data.
  
  
  RESULTS
  Only four seizure types were seen: GS, complex partial (CPS), tonic seizures (TS) and secondarily generalized tonic-clonic seizures (sGTC). An individual patient presented either CPS or TS, but not both. GS progressed to CPS or TS, but not both. Ictal patterns in GS/TS and in GS/CPS overlapped, suggesting ictal spread from the HH to other cortical regions. Ictal SPECT patterns also showed GS/TS overlap. Patients with GS-CPS presented a more benign profile with preserved cognition and clinical-EEG features of temporal lobe epilepsy. Patients with GS-TS had clinical-EEG features of symptomatic generalized epilepsy, including mental deterioration.
  
  
  CONCLUSIONS
  Video-EEG and ictal SPECT findings suggest that all seizures in HH-related epilepsy originate in the HH, with two clinical epilepsy syndromes: one resembling temporal lobe epilepsy and a more catastrophic syndrome, with features of a symptomatic generalized epilepsy. The epilepsy syndrome may be determined by HH size or by seizure spread pattern.
authors.affiliation: Hospital das Clinicas Faculdade de Medicina Universidade de Sao Paulo, Brazil, Neurology, Al. Lorena, 983 apto 82, 01424-904 Sao Paulo, Brazil. castrolh@usp.br
authors.forename: Luiz H
authors.initials: LH
authors.lastname: Castro
authors.forename: Luiz K
authors.initials: LK
authors.lastname: Ferreira
authors.forename: Leandro R
authors.initials: LR
authors.lastname: Teles
authors.forename: Carmen L
authors.initials: CL
authors.lastname: Jorge
authors.forename: Paula R
authors.initials: PR
authors.lastname: Arantes
authors.forename: Carla R
authors.initials: CR
authors.lastname: Ono
authors.forename: Carla C
authors.initials: CC
authors.lastname: Adda
authors.forename: Rosa F
authors.initials: RF
authors.lastname: Valerio
mesh_terms: D000293:Adolescent; D000328:Adult; D003072:Cognition Disorders / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D004569:Electroencephalography; D004828:Epilepsies, Partial / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D004830:Epilepsy, Tonic-Clonic / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006222:Hamartoma / Q000150:complications*; D006801:Humans; D007027:Hypothalamic Diseases / Q000150:complications*; D008297:Male; D015899:Tomography, Emission-Computed, Single-Photon; D014741:Video Recording