To determine the frequency and significance of the EEG features of hyp
sarrhythmia, we analyzed the pre-ACTH records of 53 consecutive patien
ts with infantile spasms for the severity of the following abnormaliti
es: disorganization of background, slowing, high amplitude, spike acti
vity, and for the presence or absence of each of the following pattern
s and variants: electrodecremental discharges, absence of normal sleep
activity, relative normalization, hemihypsarrhythmia, burst suppressi
on (BS), occipital hypsarrhythmia, interhemispheric asymmetry, and int
erhemispheric synchronization. We calculated a total score indicating
the severity of the hypsarrhythmia for each record. The hypsarrhythmia
variant patterns occurred frequently in up to 69% of the records. Pat
ients with cerebral dysgenesis were more likely to have hemihypsarrhyt
hmia or BS pattern persistent throughout the EEG. Patients with histor
y of perinatal hypoxia-ischemia were more Likely to have absence of no
rmal sleep activity. The occurrence of each of the other variant patte
rns did not correlate with etiology. Favorable outcome did not correla
te with the occurrence, or absence, of any of the variant patterns but
was associated with faster background activity (<75% delta), a lower
total hypsarrhythmia score (less than or equal to 10), and with absenc
e of electrodecremental discharges on the pre-ACTH EEG. We conclude th
at variant patterns of hypsarrhythmia are frequent, generally do not c
orrelate with prognosis, and thus are best included within the definit
ion of hypsarrhythmia. The severity of the hypsarrhythmia, however, do
es have significant prognostic implications.