21 new cases of infantile spasms were reported in 1976 from paediatric departments in Denmark. The connection between infantile spasms and the Lennox-Gastaut syndrome is mentioned, because of reports of a significantly higher incidence of HLA-B7 in children with Lennox-Gastaut syndrome. The HLA antigen distribution in 19 of the 21 children was compared with that of 1967 healthy adults. No diffe...

Infantile Spasms is a rare disorder consisting of epileptic spasms that occur before the age of one year. This severe epilepsy affects about 0.31 in 1000 births with an average age at onset of 6 months. Treatment usually consists of hormonal therapies (adrenocorticotropic hormone or corticosteroids) or vigabatrin. Decisions about choice of therapy are complicated because these first-line treatm...

OBJECTIVE To review the result of the infantile spasms' treatment with sodium valproate followed by nitrazepam or clonazepam. STUDY DESIGN Descriptive retrospective study. SETTING Srinagarind Hospital, Department of Pediatrics, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. MATERIAL AND METHOD Twenty-four infantile spasms admitted between January 1994 and December 2003 we...

We report the case of an 18-month-old child with infantile spasms and a hypsarrhythmic electroencephalogram (EEG) pattern associated with a porencephalic cyst. Surgical removal of the cyst and its surrounding tissue was performed following failure of medical therapy. Postoperatively, the patient has been free of infantile spasms for 12 months and the EEG has normalized. He has been maintained o...

tuberous sclerosis) (11–13). Therefore, most recently the treatment of WS with valproate has been overtaken and overlooked (14, 15). Overall, the treatment strategies in WS [either the first-line treatments (e.g., ACTH and vigabatrin) or the more classical nongolden treatments (e.g., valproate or other newer anticonvulsants)] are based on the assumption that an early initiation of therapy coupl...

Overview Infantile spasms, also known as West syndrome, is a catastrophic childhood epilepsy with seizures that are difficult to control; it is associated with mental retardation. It usually has an onset during the first year of life, and typically between 4 and 8 months. Early recognition, careful diagnostic evaluation, and proper treatment may allow some children to attain seizure control and...

The purpose of this report is to review the efficacy and safety of vigabatrin in the treatment of infantile spasms in infants suffering from tuberous sclerosis complex. We reviewed all studies published in the English-language literature investigating the use of vigabatrin in the treatment of infantile spasms. Ten studies gave results for the efficacy of vigabatrin in infantile spasms for infan...

OBJECTIVE To determine the clinical and EEG findings in children with infantile spasms at their initial presentation to the Neurophysiology Department, Children's Hospital, Lahore, Pakistan. STUDY DESIGN Observational study. PLACE AND DURATION OF STUDY The Neurophysiology Department, Children's Hospital, Lahore, Pakistan, from January 2008 to December 2010. METHODOLOGY Children aged < 24 ...

PURPOSE OF REVIEW This article reviews the most significant advances in the field of infantile spasm during the past year, with emphasis on best practise for treatment, and on some new etiological genetic and metabolic causes for the spasms, and new advances in the knowledge of tuberous sclerosis. RECENT FINDINGS Up-to-date information comparing corticotrophin, oral steroids and vigabatrin sh...