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DOI: 10.1055/s-0038-1626870
Hashimoto-Enzephalopathie – steroid-sensitive Enzephalopathie
Eine neuropsychiatrische HerausforderungHashimoto encephalopathy – steroid-responsive encephalopathy associated with autoimmune thyroiditisa neuropsychiatric challengePublication History
Eingegangen am:
05 February 2007
angenommen nach Revision am:
18 March 2007
Publication Date:
20 January 2018 (online)
Zusammenfassung
Die Hashimoto-Enzephalopathie ist eine gut behandelbare, steroid-sensitive Enzephalopathie, die sich durch eine Assoziation mit einer Autoimmun-Thyreoiditis (Hashimoto-Thyreoiditis) auszeichnet. Bis jetzt wurden ca. 110 Fallbeschreibungen der Hashimoto-Enzephalopathie in allen Altersgruppen berichtet. Unklarheit besteht über die zugrunde liegenden pathogenetischen Mechanismen. Es können zwei charakteristische Verläufe mit im Vordergrund stehenden neurologischen und psychiatrischen Symptomen unterschieden werden: eine schleichend progrediente Variante und eine akute vaskulitische Form. Trotz unterschiedlicher klinischer Präsentation findet sich generell ein gutes Ansprechen auf eine immunsuppressive Therapie. Bei konsequenter Behandlung ist von einer guten Prognose auszugehen. Im Rahmen des aktuellen Überblicks gehen wir unter anderem auf die zur Zeit diskutierten Ätiologiemodelle, die diagnostischen Standards und die empfohlenen Therapieschemata ein. Differenzialdiagnostische und differenzialtherapeutische Konsequenzen werden diskutiert.
Summary
Hashimoto`s encephalopathy is a steroid-responsive encephalopathy which accompanies an autoimmune thyroiditis (Hashimoto’s thyroiditis). Up to now about 110 cases in all age groups have been reported. The pathogenetic mechanism of the disease remains still unclear. Two characteristically courses with psychiatric and neurological symptoms are distinguished: an insidious diffuse progressive type and a more acute vasculitic type. Despite different clinical presentation an over all excellent response to immunosuppressive therapy was demonstrated. In this overview we summarise the recently discussed etiological models, the diagnostic hallmarks and the suggested therapeutic algorithm. Diffential-diagnostic and differential-therapeutic implications are discussed.
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