Summary: Purpose: To investigate the distinctive features of patients with West syndrome who had partial seizures followed by epileptic spasms (PS-ES).Methods: We examined 45 patients with West syndrome whose epileptic spasms were recorded with simultaneous video-electroencephalography (EEG) monitoring between 1982 and 1996. We investigated the patients who had PS-ES and compared the PS-ES patients with the 37 patients without Results: Of the 45 patients who had epileptic spasms in clusters (ES) and hypsarrhythmia on the interictal EEG, eight (1 7%) had ES preceded by partial seizures (PS) with a close temporal association. Seven of these eight were female patients. The underlying disorders were tuberous sclerosis (one patient), Aicardi syndrome (one), nonketotic hyperglycinemia PS-ES.(one), and focal cortical dysplasia (one). The etiology was unknown in the remaining four patients, but was suspected to be of prenatal origin in three. Three types of seizure sequence were identified: PS followed several seconds later by ES (two patients), alternating PS and ES starting with PS (three), and PS gradually replaced by ES with overlapping of the two (three). PS-ES disappeared or was replaced by other types of seizures in 1-34 months. Six patients could not walk, and all patients could not speak any sentences at age 3 years.Conclusions: Compared with patients without PS-ES, those with PS-ES more often had organic brain lesions of prenatal origin, other types of seizures before the onset of ES, asymmetric hypsarrhythmia on the EEG, and poor psychomotor outcome. Key Words: West syndrome-Partial seizureEpileptic spasms-Development-Infant.West syndrome is characterized by certain types of seizures [i.e., epileptic spasms (ESs)], which often occur in clusters. However, some patients with this epileptic syndrome have other types of seizures, especially partial seizures (PSs), either dependent on or independent of ES (1-13). An increasing number of reports on the temporal association of ESs and PSs have been published (4-6,8-10,12,13). Hrachovy et al. (1 1) stated that the coupling of PSs with ESs occurs rarely and in some instances is explained by chance coincidence. Other investigators, however, reported much higher incidences of ES, which were closely associated with PS in West syndrome patients (1 0,12,13). To elucidate this seizure sequence and the relation between ESs and PSs in West syndrome, we retrospectively reviewed patients who had hypsarrhythmia on interictal EEG and ESs evidenced by simultaneous video-EEG monitoring (SVEM).Accepted April 12, 1999. Address correspondence and reprint requests to Dr. T. Kubota at Department of Pediatrics, Nagoya University School of Medicine, 65 Tsurumai, Showa-ku, Nagoya, Japan, 466-8550.
METHODS AND SUBJECTSBetween 1982 and 1996, ES was recorded at least once in 45 patients with SVEM at Nagoya University Hospital. They all had hypsarrhythmia on the interictal EEG and were admitted for the treatment of the epileptic seizures. After admission, all of the patients underwent a full ...