OBJECTIVES To assess the seizure and developmental outcome in children with West syndrome with respect to treatment lag. METHODS Twenty-six children satisfying inclusion criteria of West syndrome i.e., infantile spasms, psychomotor retardation and abnormal EEG pattern were prospectively evaluated. Response to treatment was assessed based on seizure control, EEG, developmental assessment and p...

Background: Considering the inadequacies of current therapeutic regimens for infantile spasms (IS), and the frequent and serious side effects of Some regimens, the ongoing search for more enhanced protocols is understandable. Materials and Methods: We have compared the therapeutic and adverse effects of vitamin B6 given in high doses with those of prednisolone in a randomized controlled clinica...

Infantile spasms is an epileptic encephalopathy of early infancy with specific clinical and electroencephalographic (EEG) features, limited treatment options, and a poor prognosis. Efforts to develop improved treatment options have been hindered by the lack of experimental models in which to test prospective therapies. The neuropeptide adrenocorticotropic hormone (ACTH) is effective in many cas...

Abstract Background Epilepsy is a chronic debilitating disease especially in pediatric population. Most of reported studies for corpus callosotomy as palliative surgery drug-resistant cases are on limited number and there scarcity literature outcomes from developing countries. Here, we present our study large series with analysis potential predicators the era more expensive devices like vagal n...

The immediate effects of the use of corticotrophin or cortisone in patients with infantile spasms may be dramatic (Sorel and Dusaucy-Bauloye, 1958; Low, 1958; Sorel, 1959), but the more permanent results are less easily assessed. There is still no confirmation of Sorel's claims that corticotrophin can produce prolonged benefit, in particular mental improvement, for long-term improvement can occ...

background & importance: primary hemifacial spasm is usually caused by microvascular compression of the facial nerve at its root exit zone at brainstem without any space-occupying pathology. secondary or so-called symptomatic hemifacial spasm has an additional underlying pathology e.g. tumors leading to the microvascular compression. we review and discuss the pathophysiology and the literature ...

Down syndrome (DS) is the most common genetic cause of mental retardation. It is estimated that 5-13% of persons affected by DS have seizures. Infantile spasms are the most common type of seizures and usually are well controlled with steroids and antiepileptic drugs. We present 11 children at the age of 3 years and 4 months to 10 years and 7 months with DS and infantile spasms, treated at Child...

Background Tuberous Sclerosis Complex (TSC), and Spinal Muscular Atrophy (SMA) are two inherited disorders while they are genetically independent. TSC is characterized by the formation of multiple hamartomas in nearly all organs. SMA is a destructive neurological disorder leading to progressive muscular weakness and atrophy. Case Presentation</e...

We report on a female patient with severe infantile spasms, profound global developmental arrest, hypsarrhythmia and severe mental retardation, associated with a de novo apparently balanced X;autosome translocation. Her neurological phenotype resembles that of X-linked infantile spasms (ISSX). Molecular study showed that the translocation disrupts a transcript involved in GTPases signalling, IQ...