Interictal cerebral blood flow abnormality in cryptogenic West syndrome

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Shin-ichiro Hamano1, Norimichi Higurashi, Reiko Koichihara

  • 1Division of Neurology, Saitama Children's Medical Center, Saitama, Japan.

Epilepsia
|February 6, 2010
PubMed

Insights

West syndrome infants show widespread cerebral hypoperfusion, particularly in posterior brain regions, indicating transient brain dysfunction. This early hypoperfusion, especially in the hippocampus, may predict future cognitive impairment in West syndrome.

Area of Science:

  • Neurology
  • Pediatrics
  • Radiology

Background:

  • West syndrome is a severe form of epilepsy presenting in infancy.
  • Understanding the underlying cerebral blood flow abnormalities is crucial for early diagnosis and management.
  • Interictal regional cerebral blood flow (rCBF) patterns in West syndrome at onset are not fully understood.

Purpose of the Study:

  • To investigate interictal regional cerebral blood flow (rCBF) abnormalities in infants with cryptogenic West syndrome at the onset of the condition.
  • To identify specific brain regions affected by altered rCBF.

Main Methods:

  • Quantitative measurement of rCBF using N-isopropyl-((123)I) p-iodoamphetamine SPECT in 14 infants with cryptogenic West syndrome.
  • Automated region of interest (ROI) analysis segmented the brain into 12 areas.
  • Comparison of rCBF between patients and age-matched controls with focal epilepsy.

Main Results:

  • Cryptogenic West syndrome exhibited significant cerebral hypoperfusion compared to controls, despite visually normal SPECT scans.
  • Hypoperfusion was observed bilaterally in central, posterior cerebral, pericallosal, lenticular nucleus, and hippocampus, and unilaterally in parietal, temporal, cerebellum, angular, and thalamus segments.
  • No significant differences in rCBF were found based on the duration from onset to SPECT imaging.

Conclusions:

  • Widespread cerebral hypoperfusion with posterior predominance, including the hippocampus and lenticular nucleus, suggests transient, widespread cerebral dysfunction in West syndrome.
  • Hippocampal hypoperfusion may indicate dysfunction within the brain-adrenal axis circuitry.
  • This early cerebral dysfunction could be linked to hypsarrhythmia, epileptic spasms, and potential subsequent cognitive impairment in West syndrome.
Abstract

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