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 diag...

Hypsarhythmia was originally spelled with one 'r' by Drs Frederick and Erna Gibbs when they coined the term in 1952.

West syndrome is a generalized epileptic syndrome of infancy, that is characterized by clinical spasms and hypsarrhythmia and usually has an onset age before two years old. Children affected by West syndrome have a high risk of severe psychomotor developmental delay. Infants with West syndrome without identification of underlying neurological disorder after adequate investigations are classifie...

Infants with West Syndrome and underlying structural pathology typically experience persistent symptomatic focal seizures and intellectual disability. We performed a retrospective case review of 84 patients with West Syndrome evaluated at one institution between 1990 and 2013. From this group we identified three patients with West syndrome and congenital hemiplegia who later developed genetic e...

The aim of the study was to evaluate magnetic resonance imaging (MRI) findings in infants with periventricular leukomalacia (PVL) and West syndrome (WS) and determine the neurodevelopmental outcome in children with West syndrome and PVL. Ultrasound and brain MRI were performed in 37 infants with recognized PVL. PVL was categorized according to De Vries, whereas West syndrome was categorized acc...

Symptomatic West syndrome has heterogeneous backgrounds. Recently, two novel genes, ARX and CDKL5, have been found to be responsible for cryptogenic West syndrome or infantile spasms. Both are located in the human chromosome Xp22 region and are mainly expressed and play roles in fetal brain. Moreover, several genes responsible for brain malformations including lissencephaly, which is frequently...

The ketogenic diet has been in use for the last 90 years, and its role in the treatment of epilepsy in the pediatric population has been gaining recognition. It can be helpful in many types of epilepsies, even the more severe ones, and has a beneficial effect on the child's alertness and cognition, which can be impaired by both the condition and the medications needed for controlling it. Parent...

UNLABELLED Management of West syndrome is unsatisfactory. In our clinic we observed that a significant proportion of patients respond to usual dose of valproate. OBJECTIVE To prospectively assess the efficacy of valproate in controlling infantile spasms in West syndrome. METHODS Consecutive patients presenting with West syndrome to the Pediatric Neurology Clinic or general outpatient depart...