Abstract
Objective We evaluated the electroclinical features, etiology, treatment, and outcome of 16
patients with single-epileptic spasms (ESs) with or without hypsarrhythmia (WoH).
Methods Nine boys and seven girls had single-ESs. ESs were considered as single epileptic
spasm variants when no other spasm occurred for 1 minute before and after each spasm.
Age at the onset of ESs was between 2 and 84 months, with a mean age of 11 months.
Results We recognized a group of 15 patients with single-ESs as the main type of seizure;
6 patients with WoH and 9 patients with hypsarrhythmia, respectively. Nine of these
15 patients had other types of seizures before the onset of single-ESs, and 12 patients
had other types of seizures during the period in which the ESs occurred. Nine of 15
patients had a structural and seven had an unknown etiology. In 10 cases, the ESs
were refractory to antiepileptic drugs, while 4 patients responded well to adrenocorticotropic
hormone (ACTH), 1 to pyridoxine, and 2 to the ketogenic diet (KD). The remaining patient
(patient.16) had single-ESs and electroclinical features of Lennox–Gastaut syndrome
(LGS).
Conclusion In this article, we present a series of infants who had daily single-ESs with or
WoH. Those with single-ESs with hypsarrhythmia evolved to an epileptic encephalopathy.
Video-electroencephalogram (EEG) and polygraphic-EEG recordings are crucial to identify
the single-ESs.
Keywords
infantile - epileptic spasms - hypsarrhythmia - single
