K. Haginoya et al., The perfusion defect seen with SPECT in West syndrome is not correlated with seizure prognosis or developmental outcome, BRAIN DEVEL, 22(1), 2000, pp. 16-23
We used interictal single photon emission computed tomography (SPECT) on 40
patients with West syndrome to determine whether cortical perfusion abnorm
alities are closely related to the development of West syndrome and whether
they are correlated with the long-term seizure prognosis or the developmen
tal outcome. Localized cortical perfusion abnormalities were seen in 24 pat
ients (60%), while 15 patients (38%) were classified as normal. The remaini
ng patient showed hyperperfusion of the basal ganglia bilaterally. Of 24 pa
tients with localized perfusion abnormalities, unifocal cortical hypoperfus
ion was present in 11, multifocal hypoperfusion in 10, multiple cortical hy
po- and hyperperfusion in one, hyperperfusion of the bilateral frontal cort
ices and brain stem in one, end fecal hyperperfusion in the residual fronta
l cortex in one. For statistical analysis, we focused on 26 patients (crypt
ogenic; 10, symptomatic; 16), who were followed for more than 2 years after
the onset of tonic spasms (mean 5.0 years). The results showed that focal
cortical perfusion abnormalities were not correlated with the rang-term sei
zure prognosis, the developmental outcome, or the response to ACTH therapy,
In agreement with previous reports, the results of interictal SPECT sugges
ted that focal cortical lesions play an important role in the development o
f West syndrome. However, statistical analysis showed that the existence of
cortical dysfunction as defined by SPECT did not predict the seizure progn
osis or the developmental outcome. (C) 2000 Elsevier Science B.V. All right
s reserved.