Miller-Fisher-Syndrom und Bickerstaff-Enzephalitis: Das Anti-GQ1b-Syndrom: 2 Seiten einer Medaille

Zusammenfassung

Historisch wurden das Miller-Fisher-Syndrom (MFS) und die Bickerstaff-Hirnstammenzephalitis (BBE) aufgrund ihrer Unterschiede in der Beteiligung des peripheren und zentralen Nervensystems lange Zeit als grundsätzlich voneinander verschiedene Krankheitsentitäten betrachtet. Analysen jüngerer Daten größerer Patientenkohorten im Hinblick auf klinische Zeichen, Krankheitsverläufe, neurophysiologische Untersuchungen und neuroradiologische Befunde sowie vor allem die Untersuchung immunpathogenetischer Faktoren stellen diese klassische nosologische Einordnung jedoch in Frage: MFS und BBE bilden Teile eines kontinuierlichen, durch gemeinsame autoreaktive Antikörper gekennzeichneten Spektrums inflammatorischer Erkrankungen mit variabler Beteiligung von peripherem und zentralem Nervensystem. Die Arbeit präsentiert klinische Charakteristika, pathophysiologische Erkenntnisse und therapeutische Implikationen dieses Erkrankungsspektrums.

Abstract

Miller Fisher syndrome (MFS) and Bickerstaff’s brainstem encephalitis (BBE) have long been regarded as mutually exclusive disorders of the peripheral (PNS) and central nervous system (CNS), respectively. Recent studies analysing the pathogenesis, course, signs and symptoms of both diseases in large numbers of patients as well as neurophysiological and imaging data contradict this historical nosological classification. MFS and BBE share a common immunopathogenesis and form closely related parts of a continuous spectrum of inflammatory disorders variably involving PNS and CNS. We here present an overview of clinical characteristics, recent pathophysiological findings and therapeutic implications of this disease spectrum.

Schlüsselwörter

Miller-Fisher-Syndrom - Bickerstaff-Enzephalitis - Anti-GQ1b-Antikörper

Keywords

miller-fisher syndrome - bickerstaff brainstem encephalitis - anti-GQ1b antibodies

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