Long-term Outcome of Cognitive Development in Patients with Cryptogenic West Syndrome

DOI Web Site 14 References
  • Koichihara Reiko
    Division of Child Health and Human Development, Saitama Children's Medical Center (SCMC)
  • Hamano Shin-ichiro
    Division of Neurology, Saitama Children's Medical Center (SCMC)
  • Ikemoto Satoru
    Division of Child Health and Human Development, Saitama Children's Medical Center (SCMC)
  • Hiwatari Erika
    Division of Neurology, Saitama Children's Medical Center (SCMC)
  • Hirata Yuko
    Department of Pediatrics, Tokyo Metropolitan Kita Medical and Rehabilitation Center
  • Matsuura Ryuki
    Division of Neurology, Saitama Children's Medical Center (SCMC)
  • Minamitani Motoyuki
    Division of Neurology, Saitama Children's Medical Center (SCMC)

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  • 潜因性ウエスト症候群における知能予後良好例の臨床経過

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<p>To evaluate the characteristics of patients with cryptogenic West syndrome who exhibited favorable long-term cognitive outcomes (10 patients), we retrospectively investigated the patients' information, their responses to initial treatments and during subsequent clinical courses, including epileptic seizures, findings of electroencephalography and cognitive developments. These outcomes were compared with those of patients exhibiting moderately delayed (5 patients) and severely delayed cognitive outcome (10 patients). The differences in short treatment lag between favorable and severely delayed group was statistically significant. All the patients in the favorable outcome group responded to initial treatment or ACTH therapy and showed normal development after 6 months of the initial treatment. Although development was delayed in a few cases not long after initial treatment, they caught up with the normal range up to that point. These seemed highly likely prognostic characteristics of patients with favorable cognitive outcomes. On the other hand, there were some cases where delayed initial treatment could not make up for favorable outcome even though they sustained response to the treatment and absence of EEG abnormalities after initial treatment. In order to achieve a favorable cognitive outcome, it is necessary to not disturb the formation of neuronal networks during this period of early infancy.</p>

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