While >200 types of etiologies have been shown to be involved in the pathogenesis of infantile spasms, the pathophysiology of infantile spasms remains largely elusive. Pre-natal stress and hypothalamic-pituitary-adrenal axis dysfunction were shown to be involved in the development of infantile spasms. To test the genetic association between the CRHR1 gene, which encodes the corticotrophin-relea...

AIMS Infantile spasms are usually resistant to conventional antiepileptic drugs. Although adrenocorticotropic hormones or vigabatrin are regarded as standard agents for the treatment of infantile spasms, there are still limitations of their use. We determined the efficacy and tolerability of high-dose zonisamide in patients with recent-onset infantile spasms. METHODS Seventeen patients with i...

A 7-month-old boy with glutaric aciduria type 1 (GA1) presented with 1 week of clustered flexor spasms. Examination revealed mild axial hypotonia without encephalopathy. Video-EEG monitoring revealed hypsarrhythmia and infantile spasms (figure, A). MRI showed acute basal ganglia injury (figure, B). After 3 weeks of prednisolone treatment, 5-month follow-up showed continued resolution of hypsarr...

Background: West syndrome (WS) is an epileptic encephalopathy characterized by spasms, neurodevelopment delay, and hypsarrhythmia on electroencephalography. Infantile spasms have incidence of about 0.43 per 1000 livebirths occur between 3 12 months age. The drug's choice the adrenocorticotrophic hormone (ACTH), with varied response rates limited efficacy data, relapses, evolution to other kinds...

Objective: To update the 2004 American Academy of Neurology/Child Neurology Society practice parameter on treatment of infantile spasms in children. Methods: MEDLINE and EMBASE were searched from 2002 to 2011 and searches of reference lists of retrieved articles were performed. Sixty-eight articles were selected for detailed review; 26 were included in the analysis. Recommendations were based o...

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...

PURPOSE Infantile spasms is a severe infantile seizure disorder. Several factors affect developmental outcome, especially the underlying etiology of the spasms. Treatment also affects outcome. Both age at onset of spasms and lead time to treatment (the time from onset of spasms to start of treatment) may be important. We investigated these factors. METHODS Developmental assessment using Vinel...

West syndrome (infantile spasms) is an epileptic encephalopathy that includes psychomotor deterioration. In rare cases, it is due to an inherited, progressive metabolic disease. More than 25 inborn errors of metabolism have been considered etiologic or predisposing factors for infantile spasms. This is a review of the literature on reported cases of children diagnosed with a metabolic disease w...

We present the case of a 50-year-old female patient with Friedreich ataxia (FA) who was treated successfully with an intrathecal baclofen (ITB)-delivering pump for painful spasms. To our knowledge, this is the second reported case of FA where ITB relieved painful and disabling spasms. We suggest that ITB should be considered in the treatment of disabling spasms in patients with FA.

Background: Brachial artery spasms may occur during transradial cardiac catheterization. The purpose of this study was to examine the relationship between brachial artery spasms after transradial cardiac catheterization and coronary artery disease. Methods: Two hundred and forty-one patients who received transradial cardiac catheterization for diagnosis of coronary artery disease were enrolled ...