west syndrome in south iran: electro-clinical manifestations

how to cite this article: asadi-pooya aa, sharifzade m. west syndrome in south iran: electro-clinical manifestations. iran j child neurol. 2013 summer; 7(3): 40-44. objective we aimed to determine the clinical and electroencephalographic (eeg) characteristics of the patients with west syndrome (ws) in south iran. materials & 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. conclusion 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.   references 1. blume wt, lüders ho, mizrahi e, tassinari c, van emde boas w, engel j jr. glossary of descriptive terminology for ictal semiology: report of the ilae task force on classification and terminology. epilepsia. 2001 sep;42(9):1212-8. 2. carmant l. infantile spasms: west syndrome. arch neurol. 2002 feb;59(2):317-8. 3. hrachovy ra. west’s syndrome (infantile spasms).clinical description and diagnosis. adv exp med biol.2002;497:33-50. review. 4. riikonen r, donner m. incidence and aetiology of infantile spasms from 1960 to 1976: a population study in finland. dev med child neurol. 1979 jun;21(3):333-43. 5. dulac o. what is west syndrome? brain dev. 2001 nov;23(7):447-52. review. 6. wong v. west syndrome-the university of hong kong experience (1970-2000). brain dev. 2001 nov;23(7):609-15. 7. kalra v, gulati s, pandey rm, menon s. west syndrome and other infantile epileptic encephalopathies -indian hospital experience. brain dev. 2001 nov; 23(7):593-602. corrected and republished in: brain dev. 2002 mar;24(2):130-9. 8. young c; taiwan child neurology society. national survey of west syndrome in taiwan. brain dev. 2001 nov;23(7):570-4. 9. asadi-pooya aa, mintzer s, sperling mr. nutritional supplements, foods, and epilepsy: is there a relationship?epilepsia. 2008 nov;49(11):1819-27. 10. mikati ma, trevathan e, krishnamoorthy ks, lombroso ct. pyridoxine - dependent epilepsy: eeg investigations and long-term follow-up. electroencephalogr clin neurophysiol. 1991 mar;78(3):215-21. 11. nabbout r, soufflet c, plouin p, dulac o. pyridoxine dependent epilepsy: a suggestive electroclinical pattern. arch dis child fetal neonatal ed. 1999 sep;81(2):f125-9. 12. gospe sm jr. pyridoxine-dependent seizures: new genetic and biochemical clues to help with diagnosis and treatment. curr opin neurol. 2006 apr;19(2):148-53. review. 13.riikonen r. the latest on infantile spasms. curr opin neurol. 2005 apr;18(2):91-5. review.