Regional cerebral blood flow and developmental outcome in cryptogenic West syndrome

To elucidate the relation between alterations of regional cerebral blood flow (rCBF) by adrenocorticotropic hormone (ACTH) therapy and developmental outcomes of cryptogenic West syndrome.

Quantitative measurement of rCBF, with autoradiography method using N-isopropyl-((123)I) p-iodoamphetamine single photon emission computed tomography before and after ACTH therapy, was performed on 17 infants with cryptogenic West syndrome. Regions of interest for rCBF were placed bilaterally in the cerebellum, the thalamus, the caudate nucleus, and the frontal, temporal, and occipital cortices. RCBFs and the alteration ratios calculated from rCBFs before and after ACTH therapy were compared between two groups: the normal and delayed groups, which were divided by developmental outcome at 2 years old.

RCBFs before the therapy were not different statistically between the normal and delayed groups, and between those groups and the control group also. RCBFs after ACTH therapy of the occipital, thalamic and cerebellar regions were different between the normal and delayed groups (p < 0.05). Alteration ratios were different between the normal and delayed groups, in all of the regions but the frontal region (p < 0.05).

This study showed the differences of rCBF response by ACTH therapy between the normal and delayed groups of cryptogenic West syndrome. The difference of rCBF alteration might be associated with maturation of the cerebrovascular system, or influence of corticotropin-releasing hormone regarding the brain-adrenal-axis.