Focal cortical disturbances are frequent sequelae in West syndrome (WS
) even though it is a generalized epileptic syndrome. Functional neuro
imaging was used to determine whether focal perfusion abnormalities ex
ist at WS onset and change during evolution. We studied regional cereb
ral blood flow (rCBF) at different stages of WS. Mean CBF (mCBF) and r
CBF were measured using SPECT (single photon emission computed tomogra
phy) and Xe-133 in 13 WS patients: at onset (20 cases), just after ste
roids (17 cases), and after a mean follow-up of 2 years (26 cases). At
WS onset, interictal mCBF was increased as the result of foci of hype
r- and hypoperfusion, which were, respectively, mainly located in the
frontal and posterior cortex. Just after steroid therapy, mCBF decreas
ed without any focal predominance. During follow-up, hypoperfused foci
remained unchanged whereas the frontal hyperperfused foci decreased a
fter spasm control. Our results show that focal abnormalities are pres
ent at WS onset. Focal hypoactivity could reflect a cortical lesion re
sponsible for WS and focal hyperactivity could play a role in the pers
istence of generalized epilepsy.