title: Early-onset benign occipital seizure susceptibility syndrome.
abstract: OBJECTIVE
  Childhood epilepsy with occipital paroxysms (CEOP) is characterised by ictal visual hallucinations and occipital epileptiform activity on interictal EEG. A variant has been described with nonvisual symptoms including tonic head and eye deviation, vomiting, and episodes of partial status epilepticus. We fully documented the electroclinical features of such patients to determine whether classification separate from CEOP is justified.
  
  
  METHODS
  This was a multicentre study with participating investigators submitting details of patients with idiopathic occipital seizures characterised by ictal head or eye deviation and vomiting.
  
  
  RESULTS
  One hundred thirteen patients were recruited. Seizures began in early childhood (mean, 4.6 years) and occurred infrequently (mean total seizures, 3); 30% of patients had only a single seizure. Two thirds of seizures were nocturnal. Ictal eye deviation occurred in 79%, vomiting in 70%, and head deviation in 35%. Seizures were predominantly complex partial in type. Partial status epilepticus occurred in 44% of patients. Seventy-four percent of patients had occipital interictal EEG epileptiform activity, predominantly right sided, with fixation-off sensitivity. Extraoccipital EEG abnormalities occurred in 35% of patients. Prognosis was excellent: the mean duration of active seizures was 1 year.
  
  
  CONCLUSIONS
  Although the two groups shared identical EEG features, the distinct clinical symptoms probably justify separate classification. Early-onset benign occipital seizure syndrome (EBOSS) is suggested as an appropriate name for the variant group.
authors.forename: C D
authors.initials: CD
authors.lastname: Ferrie
authors.forename: A
authors.initials: A
authors.lastname: Beaumanoir
authors.forename: R
authors.initials: R
authors.lastname: Guerrini
authors.forename: S
authors.initials: S
authors.lastname: Kivity
authors.forename: F
authors.initials: F
authors.lastname: Vigevano
authors.forename: Y
authors.initials: Y
authors.lastname: Takaishi
authors.forename: K
authors.initials: K
authors.lastname: Watanabe
authors.forename: L
authors.initials: L
authors.lastname: Mira
authors.forename: G
authors.initials: G
authors.lastname: Capizzi
authors.forename: P
authors.initials: P
authors.lastname: Costa
authors.forename: D
authors.initials: D
authors.lastname: Valseriati
authors.forename: D
authors.initials: D
authors.lastname: Grioni
authors.forename: P
authors.initials: P
authors.lastname: Lerman
authors.forename: S
authors.initials: S
authors.lastname: Ricci
authors.forename: P
authors.initials: P
authors.lastname: Vigliano
authors.forename: A
authors.initials: A
authors.lastname: Goumas-Kartalas
authors.forename: K
authors.initials: K
authors.lastname: Hashimoto
authors.forename: R O
authors.initials: RO
authors.lastname: Robinson
authors.forename: C P
authors.initials: CP
authors.lastname: Panayiotopoulos
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D002648:Child; D015897:Comorbidity; D003937:Diagnosis, Differential; D004569:Electroencephalography; D004827:Epilepsy / Q000145:classification / Q000175:diagnosis / Q000503:physiopathology*; D017029:Epilepsy, Complex Partial / Q000175:diagnosis / Q000503:physiopathology; D005260:Female; D006212:Hallucinations / Q000175:diagnosis / Q000453:epidemiology; D006801:Humans; D008297:Male; D009778:Occipital Lobe / Q000503:physiopathology*; D015835:Ocular Motility Disorders / Q000175:diagnosis / Q000453:epidemiology; D011379:Prognosis; D013226:Status Epilepticus / Q000175:diagnosis / Q000503:physiopathology; D014796:Visual Perception; D014839:Vomiting / Q000175:diagnosis / Q000453:epidemiology