Mechanism of tonic spasms in West syndrome viewed from ictal SPECT findings

Kazuhiro Haginoya, Mitsutoshi Munakata, Hiroyuki Yokoyama, Rie Kato, Soichiro Tanaka, Mieko Hirose, Mamiko Ishitobi, Kimiya Kon, Yasushi Yoshihara, Masaru Takayanagi, Tetsuro Yamazaki, Kazuie Iinuma

Research output: Contribution to journalArticlepeer-review

21 Citations (Scopus)

Abstract

To clarify the pathophysiology of tonic spasms, 21 patients with West syndrome were analyzed using ictal and interictal single photon emission computed tomography (SPECT). We focused on whether ictal perfusion changes were observed in the focal cortical region. Eight of the patients studied showed definite focal cortical ictal hyperperfusion, indicating that there is a unique subset of West syndrome that can be classified as infantile localization-related epilepsy. Of those eight patients, only two showed asymmetric spasms, suggesting that seizure symptomatology in infants gives only limited information on the localization-related nature of epilepsy. Furthermore, the activation of subcortical structures by focal cortical regions might be attributable to the symmetric seizure phenomena. Thirteen patients showed a diffuse pattern in their ictal SPECTs; this probably included patients with diffuse hyperperfusion and those with no changes. The following have yet to be determined: (1) whether West syndrome is divided into subgroups based on the origin of spasms, in that some patients have the origin in the cortical hemisphere and some have the origin in structures other than the cortical hemisphere, such as the brain stem; (2) whether differences in ictal SPECT patterns reflect a unique nature of tonic spasms in West syndrome, where tonic spasms appear in clusters and the interval of each spasm is different among each patient.

Original languageEnglish
Pages (from-to)496-501
Number of pages6
JournalBrain and Development
Volume23
Issue number7
DOIs
Publication statusPublished - 2001

Keywords

  • Asymmetric spasms
  • Hypsarrhythmia
  • Ictal single photon emission computed tomography
  • Infantile spasms
  • Single photon emission computed tomography
  • Tonic spasms
  • West syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Clinical Neurology

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