title: Unique Characteristics of the Photoparoxysmal Response in Patients With Neuronal Ceroid Lipofuscinosis Type 2: Can EEG Be a Biomarker?
abstract: The objective was to identify unique features of the photoparoxysmal response seen in patients with neuronal ceroid lipofuscinosis type 2 as compared to patients with a photoparoxysmal response associated with other epilepsy syndromes.
  
  
  
  Electroencephalograms from patients with neuronal ceroid lipofuscinosis type 2 seen at the authors' institution in the past 10 years as well as electroencephalograms (EEGs) reported to have a photoparoxysmal response during a single year were reviewed.
  
  
  
  A photoparoxysmal response was seen in 60% of the patients with neuronal ceroid lipofuscinosis type 2. This was most commonly seen with low frequency intermittent photic stimulation (76%) which often occurred in a time-locked fashion (63%) and was seen on the patient's initial EEG (78%). A unique pattern the authors called "sentinel" discharge was identified in 30% of EEGs in patients with neuronal ceroid lipofuscinosis.
  
  
  
  Photoparoxysmal responses in patients with neuronal ceroid lipofuscinosis type 2 have features which are distinguishing from photoparoxysmal responses seen in other epilepsies.
authors.affiliation: Nationwide Children's Hospital/Ohio State University, Division of Pediatric Neurology, Columbus, OH, USA dara.albert@nationwidechildrens.org.
authors.forename: Dara V
authors.initials: DV
authors.lastname: Albert
authors.affiliation: Biostatistics Core of Nationwide Children's Hospital, Columbus, OH, USA.
authors.forename: Han
authors.initials: H
authors.lastname: Yin
authors.affiliation: Nationwide Children's Hospital/Ohio State University, Division of Pediatric Neurology, Columbus, OH, USA.
authors.forename: Emily C
authors.initials: EC
authors.lastname: De Los Reyes
authors.affiliation: Nationwide Children's Hospital/Ohio State University, Division of Pediatric Neurology, Columbus, OH, USA.
authors.forename: Jorge
authors.initials: J
authors.lastname: Vidaurre
mesh_terms: D000293:Adolescent; D001921:Brain / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004832:Epilepsy, Absence / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D005260:Female; D006801:Humans; D009472:Neuronal Ceroid-Lipofuscinoses / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology*; D010775:Photic Stimulation; D010787:Photosensitivity Disorders / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D012189:Retrospective Studies
keywords: electroencephalogram (EEG); neuronal ceroid lipofuscinosis; pediatric epilepsy; photic stimulation; photoparoxysmal response