Zentrale Okulomotorikstörungen: klinische Diagnose, anatomische Zuordnung, Syndrome und zugrunde liegende Erkrankungen

Zusammenfassung

Der Schlüssel zur Diagnose von Augenbewegungsstörungen ist eine systematische klinische Untersuchung aller Arten von Augenbewegungen. Diese ist wie ein Fenster in Hirnstamm und Kleinhirn, selbst wenn die Bildgebung unauffällig ist. Die Untersuchung umfasst die folgenden Aspekte: Augenposition, Untersuchung auf einen Spontannystagmus, Motilität, Blickfolge, Blickhaltefunktion, Sakkaden, Vergenzreaktion, optokinetischer Nystagmus, Funktion des vestibulookulären Reflexes (VOR) sowie die Fixationssuppression des VOR. Anatomisch relevante Strukturen sind Mesenzephalon, Pons, Medulla oblongata, Zerebellum und sehr selten der Kortex. Topografisch-anatomisch gelten die folgenden einfachen klinischen Regeln: vertikale und torsionelle Augenbewegungen werden vorwiegend im Mesenzephalon (relevante Strukturen sind der rostrale interstitielle Kern des Fasciculus longitudinalis medialis und der interstitielle Nucleus Cajal) und horizontale Augenbewegungen im Pons (relevante Struktur ist z. B. die paramediane pontine Formatio reticularis) generiert. Somit finden sich z. B. bei Mittelhirnläsionen eine vertikale Sakkaden- oder Blickparese und ein isolierter vertikaler Blickrichtungsnystagmus und bei Läsionen im Bereich des Pons entsprechende horizontale Störungen sowie eine internukleäre Ophthalmoplegie. Läsionen der lateralen Medulla oblongata (Wallenberg-Syndrom) führen zu typischen Befunden mit Ocular Tilt Reaction, Fixationsnystagmus und Sakkadendysmetrie. Das Zerebellum spielt eine Rolle bei praktisch allen Augenbewegungen; typische klinische Zeichen sind eine allseitige Blickfolgesakkadierung, Blickrichtungsnystagmus oder dysmetrische Sakkaden. Wichtig für die Beurteilung der zugrunde liegenden Erkrankungen ist der Verlauf: akut bei Infarkten, subakut bei entzündlichen Erkrankungen, Thiaminmangel oder unerwünschter Medikamentenwirkung sowie chronisch progredient bei hereditären Erkrankungen wie Niemann-Pick Typ C mit typischer, zunächst vertikaler und dann horizontaler Sakkadenparese oder neurodegenerativen Erkrankungen wie der progressiven supranukleären Blickparese. Die Behandlung hängt von der zugrunde liegenden Erkrankung ab. Abschließend: in einem begleitenden Artikel werden in dieser Zeitschrift die häufigsten und klinisch relevanten Nystagmusformen wie z. B. Downbeat-, Upbeat-, Fixationspendelnystagmus, infantiler oder periodisch-alternierender Nystagmus und deren klinische Untersuchung dargestellt, sodass auf diese hier nicht detailliert eingegangen wird, wobei es bewusst Überlappungen gibt.

Schlüsselwörter

Blickrichtungsnystagmus - Sakkadenparese - Blickparese - sakkadierte Blickfolge - internukleäre Ophthalmoplegie

Publication History

Received: 13 September 2021

Accepted: 22 September 2021

Article published online:
16 November 2021

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