Limbische Enzephalitis

C. G. Bien

doi:10.1055/s-0038-1627325

Nervenheilkunde, Table of Contents

Nervenheilkunde 2008; 27(05): 413-420
DOI: 10.1055/s-0038-1627325

Original- und Übersichtsarbeiten - Original and Review Articles

Schattauer GmbH

Limbische Enzephalitis

Diagnostik und ManagementLimbic encephalitisDiagnosis and management

C. G. Bien

¹Universität Bonn, Klinik und Poliklinik für Epileptologie

› Author Affiliations

Abstract

Zusammenfassung

Die limbische Enzephalitis (LE) wurde in den 1960er-Jahren in klinisch-neuropathologischen Fallserien bei erwachsenen Patienten beschrieben. Zunächst wurde die paraneoplastische Form der LE identifiziert. Es wurden Autoantikörper (z. B. Hu-Antikörper) im Serum und im Liquor betroffener Patienten entdeckt, die an Hirn- und Tumorzellen binden. Diese Antikörper stellten einen erheblichen diagnostischen Fortschritt dar, erklären aber angesichts der intrazellulären Lage ihrer Antigene nicht die Pathogenese der Erkrankung. In letzter Zeit wird die zahlenmäßige Bedeutung nicht-paraneoplastischer LE-Fälle deutlich. Bei einem Teil dieser Patienten wurden Serumantikörper gegen spannungsabhängige Kaliumkanäle gefunden. Inzwischen ist auch der charakteristische MRT-Verlauf bei LE-Patienten genauer beschrieben worden: Es findet sich initial eine hippocampale Schwellung und T2-/FLAIR-Signalanhebung. Im Verlauf einiger Monate bildet sich die Schwellung zurück und geht allmählich in eine Atrophie bei anhaltender Signalanhebung über. Dieser charakteristische MRT-Verlauf erlaubt die Stellung der Diagnose einer möglichen LE auch bei Antikörper- und Tumor-negativen Patienten.

Summary

Limbic encephalitis (LE) was described in the 1960s in clinical-pathological series of adult patients. Initially, the paraneoplastic form was identified. Autoantibodies in patients’ sera were detected (e. g. Hu antibodies), which react with brain cells and tumor cells. These antibodies represented an important diagnostic progress. They do not, however, explain the pathogenesis of these syndromes. Lately, the impact of non-paraneoplastic cases has been acknowledged. In a part of these patients, serum antibodies against voltage-dependent potassium channels have been detected. Meanwhile, the characteristic MRI course of LE patients has been described: hippocampal swelling and T2-/FLAIR-signal increase are early findings. After some months, swelling regresses and hippocampal atrophy comes about with continuous signal increase. This characteristic course permits the diagnosis of possible LE also in antibody and tumor negative cases.

Schlüsselwörter

Limbische Enzephalitis - Temporallappenepilepsie - Gedächtnisstörung - Erwachsene

Keywords

Limbic encephalitis - temporal lobe epilepsy - memory disturbance - adults

Full Text

References

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