Studies on the Rett syndrome. Part 2. Polysomnographic and neuroendocrinological studies.

  • Fujino Kayo
    Department of Pediatrics, School of Medicine, University of Tokushima
  • Hashimoto Toshiaki
    Department of Pediatrics, School of Medicine, University of Tokushima

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Other Title
  • Rett症候群に関する研究 第2編: Rett症候群の睡眠ポリグラムおよび神経内分泌学的検討
  • Part 2. Polysomnographic and Neuroendocrinological Studies
  • 第2編: Rett症候群の睡眠ポリグラムおよび神経内分泌学的検討

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Abstract

The biogenesis of the Rett syndrome (RS) is unknown although there have been several reports suggesting some biochemical defect of the monoaminergic system in CNS. We performed all night polysomnography and neuroendocrinological tests in three cases of RS discovered on a survaylance study in Tokushima. The following results were obtained; 1) dissociation of sleep elements (stage 1-REM, stage 2-REM), 2) no increase in REMs/min with development, 3) abnormal patterns of BMs, TMs of each sleep stage, 4) paradoxical secretion of growth hormone (GH) and excessive secretion of prolactine in TRH provocation test, the absence of sleep enhancement of GH secretion, 5) progressive disfunction of autonomic nervous system during sleep. These results indicated that RS involves the functional disturbances of brain stem and hypothalamic monoaminergic regulatory systems.

Journal

  • NO TO HATATSU

    NO TO HATATSU 22 (1), 16-23, 1990

    THE JAPANESE SOCIETY OF CHILD NEUROLOGY

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