Chronische Enzephalitiden als Epilepsieursachen: Pathogenese, Diagnostik und Therapie

Zusammenfassung

Die folgenden entzündlich bedingten Hirnerkrankungen ohne (ersichtlichen) infektiösen Ursprung gehen häufig mit fokalen epileptischen Anfällen einher: die Rasmussen-Enzephalitis (RE), die limbische Enzephalitis (LE), die anti-N-Methyl-D-Aspartat(NMDA)-Rezeptor-Enzephalitis und die steroidresponsive Enzephalopathie mit assoziierter Autoimmunthyroiditis (SREAT, früher „Hashimoto-Enzephalopathie”). In den letzten Jahren etablieren sich pathogenetische Konzepte und diagnostische Kriterien für diese Störungen und Syndrome. Es besteht noch keine belastbare Evidenz hinsichtlich der zu bevorzugenden Therapie. Allerdings können aus Fallstudien und aus pathogenetisch basierten Überlegungen begründete Therapieempfehlungen für diese beeinträchtigenden Erkrankungen abgeleitet werden.

Abstract

The following inflammatory brain disorders without (apparent) infectious origin are frequently associated with focal epileptic seizures: Rasmussen's encephalitis (RE), limbic encephalitis (LE), anti-N-methyl-D-aspartate (NMDA) receptor encephalitis, and steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT, formerly called „Hashimoto's encephalopathy”). Pathogenetic concepts and diagnostic criteria for these disorders and syndromes are emerging. However, there is still no reliable evidence regarding the best possible therapy. In this situation, recommendations can only be derived from case studies and from pathogenetic considerations.

Schlüsselwörter

Epilepsie - Rasmussen-Enzephalitis - limbische Enzephalitis - anti-NMDA-Rezeptor-Enzephalitis - SREAT

Key words

epilepsy - Rasmussen's encephalitis - limbic encephalitis - anti-NMDA-receptor encephalitis - SREAT

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