Abstract
Here we present an update of the electroclinical features and evolution of patients
with epileptic spasms (ES) in clusters without hypsarrhythmia, with or without focal
or generalized paroxysmal discharges on the interictal electroencephalography (EEG).
Between February 1990 and July 2012, 26 patients met the electroclinical diagnostic
criteria of ES in clusters without hypsarrhythmia. The etiology of the ES was unknown
in 15 patients and structural in 11. Age at onset of the ES was between 2 and 32 mo,
with a mean age of 9 mo. Fifteen patients had seizures before the onset of ES. Focal
spikes were observed in 12 patients, bilateral spikes and spikes and waves in nine,
multifocal spikes in three, and two patients had a normal EEG. The ictal EEG recording
showed diffuse high-amplitude slow waves in 15 patients, diffuse slow waves followed
by voltage attenuation in six patients, and diffuse fast rhythms in five. ES resolved
in six patients after a mean follow-up of 9 yr. Neuropsychological development has
been normal in these six patients. Twenty patients continue with seizures refractory
to antiepileptic drugs after a mean follow-up of 13 yr. Two of them were operated;
they are seizure free, with a normal neuropsychological development. Of the 18 remaining
patients, 10 have severe mental retardation, five have moderate mental retardation,
and three have mild mental retardation. All but 14 of them show behavioral disturbances.
Our patients represent an epileptic encephalopathy that may be considered a distinct
epileptic syndrome rather than a variant of West syndrome.
Keywords
Childhood - clusters - encephalopathy - epileptic spasms - infantile spasms - hypsarrhythmia
