title: West syndrome in South Iran: electro-clinical manifestations.
abstract: OBJECTIVE
  We aimed to determine the clinical and electroencephalographic (EEG) characteristics of the patients with West syndrome (WS) in south Iran.
  
  
  METHODS
  In this retrospective study, all patients with a clinical diagnosis of WS were recruited in the outpatient epilepsy clinic at Shiraz University of Medical Sciences between September 2008 and May 2012. Age, gender, age at seizure onset, seizure type(s), epilepsy risk factors, EEG and imaging studies of all patients were registered routinely.
  
  
  RESULTS
  During the study period, 2500 patients with epilepsy were registered at our epilepsy clinic. Thirty-two patients (1.3%) were diagnosed to have WS. Age of onset (mean ± standard deviation) was 4.99 ± 3.06 months. Sixteen patients were male and 16 were female. Nine (28.1%) were reported to have two or more seizure types and 23 (71.8%) had one seizure type (epileptic spasms). At referral, no developmental delay was detected in two patients and in the rest, a mild to severe delay was noted. Electroencephalography showed typical hypsarrhythmia in 59.4% of our patients and modified hypsarrhythmia or atypical presentations were seen in 40.6%. Two patients had pyridoxine (B6)-dependent seizures, confirmed by oral B6 trial.
  
  
  CONCLUSIONS
  Variants of the classical triad of WS including other seizure types, atypical EEG findings, and normal psychomotor function at the beginning could be observed in some patients. Rarely, treatable genetic disorders (e.g., pyridoxine-dependent seizures) should be considered in those in whom no other diagnosis is evident.
authors.affiliation: Neurosciences Research Center, Shiraz University of Medical Sciences, Shiraz, Iran ; 2.Jefferson Comprehensive Epilepsy Center, Department of Neurology, Thomas Jefferson University, Philadelphia, USA.
authors.forename: Ali Akbar
authors.initials: AA
authors.lastname: Asadi-Pooya
authors.affiliation: Neurosciences Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Mohaddese
authors.initials: M
authors.lastname: Sharifzade
keywords: Electroencephalography; Spasms; West syndrome