BACKGROUND There is poor inter-rater agreement in determining the presence or absence of hypsarrhythmia among patients with infantile spasms. Yet, remission of hypsarrhythmia has been used as a clinical and research outcome measure. Two important features of hypsarrhythmia are the burden of epileptiform discharges and the amplitudes of background slow waves. We hypothesized that an electroencep...

Twenty-six children (16 boys and 10 girls) with hypsarrhythmia and infantile spasms (IS) were studied at the University of Michigan EEG Laboratory in a 4-year period. Six (2 boys, 4 girls), had asymmetric hypsarrhythmia with a preponderance of both slowing and epileptiform activity over one hemisphere. All 6 had the symptomatic form of IS, 4 with dysplastic conditions, 1 with porencephaly from ...

PURPOSE West syndrome (WS) is a severe age-related acute epileptic encephalopathy of infancy characterized by infantile spasms, hypsarrhythmia and psychomotor delay. The aim of this study was to investigate if patients with WS had an altered autonomic output to the heart. METHODS In 23 patients with WS the heart rate variability (HRV) was investigated by examining time- and frequency-domain p...

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...

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...

Hypsarhythmia was originally spelled with one 'r' by Drs Frederick and Erna Gibbs when they coined the term in 1952.

PURPOSE Hypsarrhythmia, the pathognomonic EEG pattern of West syndrome, is typically characterized by a high amplitude, arrhythmic, and asynchronous pattern. While this severely aberrant pattern would suggest severe abnormalities in connectivity, coherence has not yet been systematically assessed in hypsarrhythmia. METHODS We evaluated the EEGs of 28 infants, 12 with infantile spasms with hyp...