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Schilddrüsenhormon und Störungen des Zentralnervensystems

Thyroid hormone and disorders of the central nervous system

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Zusammenfassung

Eine Hypothyreose führt in der vulnerablen Phase der Entwicklung des Zentralnervensystems (ZNS) während der Schwangerschaft und in den ersten beiden Lebensjahren zu einer schweren mentalen und motorischen Retardierung. In Analogie zu Befunden aus Tierversuchen wird die Retardierung durch eine neuronale Migrationsstörung und eine reduzierte axonale Ausbildung verursacht. Die vorliegende Übersicht fasst die Befunde zur Behandlung einer maternen und neonatalen Hypothyreose zusammen und erläutert die aktuelle Datenlage zur Frage einer ggf. auftretenden ZNS-Störung bei nur geringer Schilddrüsenfunktionsstörung. Darüber hinaus werden seltenere Differenzialdiagnosen einer ZNS-Schädigung bei genetischen Defekten der Schilddrüsenhormonwirkung (MCT8-Defekt) und in Assoziation mit Schilddrüsenerkrankungen (Hashimoto-Enzephalopathie und NKX2.1-Gen-Defekt) besprochen.

Abstract

During the sensitive period of fetal and neonatal development, thyroid hormone deficiency leads to severe defects of the central nervous system (CNS), including mental retardation and movement defects. These clinical symptoms can be explained, based on animal studies, by a migration defect of cortical neurons as well as by reduced axonal connectivity. This review discusses the established knowledge in treating maternal and congenital hypothyroidism to avoid mental retardation. In addition, the findings on treating pregnant women and neonates with only borderline hypothyroidism are summarized. The differential diagnoses for more rare diseases of thyroid hormone resistance within the CNS (MCT8 defect), as well as defects of CNS development associated with thyroid diseases that have recently been described in children (Hashimoto’s encephalopathy and NKX2.1 deficiency syndrome), are summarized as well.

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  1. Charité-Universitätsmedizin Berlin, Campus Virchow-Klinikum, Augustenburgerplatz 1, 13353, Berlin, Deutschland

    H. Krude

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Krude, H. Schilddrüsenhormon und Störungen des Zentralnervensystems. Monatsschr Kinderheilkd 156, 961–971 (2008). https://doi.org/10.1007/s00112-008-1757-0

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  • DOI: https://doi.org/10.1007/s00112-008-1757-0

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