Abstract
In 19 cases with newly diagnosed symptomatic West syndrome, we assessed interictal regional cerebral blood flow (rCBF) before ACTH therapy with single photon emission computed tomography (SPECT) Based on the SPECT findings, we divided these cases into 3 groups: normal rCBF (Group A, 7 cases), abnormal rCBF corresponding to cerebral lesions on MRI and CT (Group B, 6 cases), and abnormal rCBF in areas different from lesions on MRI and CT (Group C, 6 cases). We compared clinical features, response to initial treatment, and short-term outcome among these 3 groups. No significant differences were found in clinical characteristics (sex, age of onset, prior seizures before onset of spasms, EEG findings). Four cases inGroup B (67%) and 5 in Group C (83%) showed complete cessation of spasms after initial treatment (high dose vitamin B6→zonisamide→ACTH therapy), while in Group A only 2 patients (29%, p>0.05;compared to Group B or Group C) responded. Although not statistically significant, short-term prognosis (both seizures and development) after a mean follow-up of 2 years and 8 months was also worst in Group A. Our results suggest that normal SPECT findings may be predictive of unfavorable prognosis in infants with symptomatic West syndrome.
