Regional Cerebral Blood Flow and Developmental Outcome in Cryptogenic West Syndrome

Abstract

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

PET in Infancy Predicts Long-Term Outcome during Adolescence in Cryptogenic West Syndrome

“…Other investigators have confirmed these results by using SPECT or PET. 10,11 In all of the previous studies, the follow-up period was limited to a minimum of 2 or 3 years, and educational outcome was not reported. Although gross motor development or simple language development can be evaluated by 2 or 3 years of age, evaluation at this age is not sufficient for higher cognitive functioning or socioeducational status.…”

“…We and others have reported PET hypometabolism or SPECT hypoperfusion during the early stage of cryptogenic West syndrome. [6][7][8][9][10][11] We also have reported evolutional changes in cortical hypometabolism on PET and have suggested the usefulness of PET for predicting the prognosis. [6][7][8][9] The results of our studies and others revealed that persistent hypometabolism or hypoperfusion after initial pharmacologic treatment suggests poor developmental outcome.…”

“…[6][7][8][9] The results of our studies and others revealed that persistent hypometabolism or hypoperfusion after initial pharmacologic treatment suggests poor developmental outcome. [6][7][8][9]11,12 However, the follow-up period of these studies was limited to a minimum of 2 or 3 years. Developmental statuses and seizures in school age or adulthood should be investigated to reveal long-term outcome.…”

Epileptic encephalopathies in infancy and early childhood

“…The following conclusions probably give a fair account of the overall prognosis irrespective of cause. 22,43,52,[69][70][71][72][73][74][75][76][77] Mortality has fallen to about 5% in developed countries because of improved medical care. Deaths may be due to the underlying cause and treatment mainly with ACTH and corticosteroids.…”

White Matter Abnormality Correlates with Developmental and Seizure Outcomes in West Syndrome of Unknown Etiology

“…2 We and others have reported hypometabolism on PET and hypoperfusion on SPECT during the early stages of cryptogenic WS. [3][4][5][6][7][8][9] Detecting these findings is useful for both predicting long-term outcomes and detecting underlying lesions. [3][4][5][6]8,9 Further progress of modern neuroimaging techniques is expected to clarify the underlying pathology in patients with WS of unknown etiology.…”