Home Contact Sitemap

eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease west syndrome
Comorbidity C0037769|west syndrome
Sentences 5
PubMedID- 23252400 west syndrome consists of infantile spasms, hypsarrhythmia, and developmental arrest.
PubMedID- 26125044 Vigabatrin (vgb; γ-vinyl-gaba; sabrilr), an antiepileptic agent that irreversibly inhibits gaba-transaminase (gaba-t) (fig.1), is an fda-approved agent employed therapeutically in infantile spasms associated with west syndrome, secondary generalized seizures, and complex partial seizures.1,2 in a study of school age children who had received vgb as infants, riikonen and coworkers3 detected visual field defects (vfd) in one third, and further verified that the rate of vfds increased from 9% to 63% as the duration of vgb treatment had increased during infancy.
PubMedID- 23397290 For example, the resolution of hypsarrhythmia in west syndrome is associated with an improvement in cognitive outcome and drives treatment choice, but the same cannot be applied to frequent inter-ictal discharges in lennox gastaut syndrome.
PubMedID- 20882816 west syndrome consists of the triad of infantile spasms, characteristic electroencephalogram (eeg) pattern of hypsarrythmia and developmental delay.
PubMedID- 25363350 Objective: fast oscillations (fos) were first explored from scalp electroencephalographic (eeg) data from hypsarrhythmia in west syndrome (infantile spasms) to investigate the meaning of fos in this epileptic encephalopathy.

Page: 1