PURPOSE To evaluate the prognostic importance of electroencephalography (EEG) findings at cessation of epileptic spasms for seizure outcome. METHODS We reviewed 71 children with West syndrome (cryptogenic 14) who had obtained control of epileptic spasms with initial treatment (adrenocorticotropic hormone (ACTH) 37, high-dose vitamin B6 2, and antiepileptic drugs 32). According to the EEG find...

OBJECTIVE Infantile spasm (IS) is a convulsive disease characterized by brief, symmetric axial muscle contraction (neck, trunk, and/or extremities). IS is a type of seizure that was first described by West in 1841, who witnessed the seizure in his own son. West's syndrome refers to the classic triad of spasms, characteristic EEG, and neurodevelopmental regression. Most cases involve flexors and...

Objectives: Among patients with West Syndrome (WS), the underlying brain dysfunction is considered to be the major determinant of cognitive outcome. However, regardless of whether the WS type is symptomatic or cryptogenic, patients with infantile spasms may have a favourable or grim prognosis. The purpose of this study was to utilize electroencephalography (EEG) to guide treatment, and to asses...

Vigabatrin was approved for the treatment of infantile spasms by the US Food and Drug Administration, but not in Japan at the time of initiating this clinical study because of concerns about irreversible peripheral visual field defects (VFDs). This study evaluated the efficacy and safety of vigabatrin for Japanese patients with infantile spasms. Of 15 patients (aged ≥4weeks and <2years) enrolle...

West syndrome is a triad of infantile spasms with hypsarrhythmia and developmental regression. It results in poor neurodevelopmental outcome due to delayed diagnosis treatment which further compounded by delay referral multidisciplinary interventions. Our case was male child west who came us at 3 years age concerns global features autism spectrum disorder. Based on thorough evaluation he starte...

Insulin-like growth factors play a key role for neuronal growth, differentiation, the survival of neurons and synaptic formation. The action of IGF-1 is most pronounced in the developing brain. In this paper we will try to give an answer to the following questions: Why are studies in children important? What clinical studies in neonatal asphyxia, infantile spasms, progressive encephalopathy-hyp...

Riboflavin transporter 1 (RFVT1) deficiency is an ultrarare metabolic disorder due to autosomal dominant pathogenic variants in SLC52A1. The RFVT1 protein mainly expressed the placenta and intestine. To our knowledge, only five cases of from three families have been reported so far. While newborns infants with SLC52A1 showed a multiple acyl-CoA dehydrogenase deficiency-like presentation, indivi...

Case presentation: A 11-month-old female patient with Trisomy 21 (Down Syndrome) who developed infantile spasms at 6-months-old. She was diagnosed West Syndrome and first treated Vigabatrin (VGB). positive response observed, control of the spams regression hypsarrhythmia electroencephalogram register. well controlled until 10-months-old, when returned developmental regression. treatment synthet...