The perfusion defect seen with SPECT in West syndrome is not correlated with seizure prognosis or developmental outcome

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.