Interictal cerebral blood flow abnormality in cryptogenic West syndrome

Abstract

SUMMARYPurpose: To elucidate the abnormality of interictal regional cerebral blood flow (rCBF) of West syndrome at the onset. Methods: Quantitative measurement of rCBF with an autoradiography method using N-isopropyl-( 123 I) p-iodoamphetamine single photon emission computed tomography (SPECT) was performed on 14 infants with cryptogenic West syndrome. Regions of interest (ROIs) for rCBF were placed automatically using an automated ROI analysis software (three-dimensional stereotactic ROI template), and were grouped into 12 segments: callosomarginal, precentral, central, parietal, angular, temporal, posterior cerebral, pericallosal, lenticular nucleus, thalamus, hippocampus, and cerebellum. We compared rCBF between the patients and seven age-matched infants with cryptogenic focal epilepsy as a control group. The patients were divided into two groups according to the duration from onset to SPECT, to compare rCBF. Results: Quantitative analysis revealed cerebral hypoperfusion in cryptogenic West syndrome with normal SPECT images under visual inspection. In bilateral central, posterior cerebral, pericallosal, lenticular nucleus, and hippocampus, and in the left parietal, temporal, and cerebellum, and in the right angular and thalamus segments there were statistical differences (p < 0.05). Compared with the duration from onset to SPECT, there were no significant differences of rCBF in all segments. Discussion: Broad cerebral hypoperfusion with posterior predominance involving the hippocampus and lenticular nucleus implies that even cryptogenic West syndrome has a widespread cerebral dysfunction at least transiently, which would correspond to clinical manifestations of hypsarrhythmia and epileptic spasms. Hippocampal hypoperfusion suggests the dysfunction of hippocampal circuitry in the brain adrenal axis, and may contribute to subsequent cognitive impairment of cryptogenic West syndrome.

Hyperactive mTOR signals in the proopiomelanocortin-expressing hippocampal neurons cause age-dependent epilepsy and premature death in mice

“…We emphasize the value of the latter hypothesis because previous studies supported this concept. For example, a clinical study using single photon emission tomography revealed decreased regional cerebral blood flow in the hippocampus of IS patients 39 . In rodents, ACTH was shown to regulate the hippocampal expressions of Crh and Crhbp 40 .…”

Involvement of the Thalamus, Hippocampus, and Brainstem in Hypsarrhythmia of West Syndrome: Simultaneous Recordings of Electroencephalography and fMRI Study

“…A previous study of interictal SPECT showed decreased perfusion of the thalamus and hippocampus in children with WS. 21 The present EEG-fMRI study suggests that epileptogenic activities during hypsarrhythmia are associated with abnormal activities in the hippocampus and thalamus and lead to dysfunction of the limbic system. This hypothesis supports the idea that early cessation of the hypsarrhythmia is important for cognitive outcomes in WS.…”