Die okuläre Neuromyotonie – klinisches Bild und Gedanken zur Pathogenese

Zusammenfassung

Die okuläre Neuromyotonie ist eine seltene, neurogen bedingte Augenbewegungsstörung. Die Patienten beklagen vorübergehende Doppelbilder infolge einer plötzlichen, schmerzlosen und unwillkürlichen Abweichung eines Auges in die Zugrichtung des hochfrequent stimulierten Augenmuskels. Das Phänomen wird in der Regel nach einer Bestrahlung beobachtet, kann aber ebenso durch eine neuronale Kompression bedingt sein. Die Pathophysiologie ist bisher nicht geklärt. Als Hypothese werden eine ephaptische Transmission, die Störung von Kaliumkanälen der neuronalen Zellmembran und eine zentrale Reorganisation diskutiert. Die Diagnose der okulären Neuromyotonie wird in der Regel anhand der klinischen Symptomatik gestellt und kann durch eine elektromyografische Untersuchung gesichert werden. Zum Ausschluss einer kompressionsbedingten Genese sollte stets eine zerebrale Bildgebung unter besonderer Beurteilung des betroffenen Hirnnervs durchgeführt werden. Die okuläre Neuromyotonie lässt sich in der Regel gut mit Carbamazepin behandeln.

Abstract

Ocular neuromyotonia (ONM) is a rare neurogenic disorder of ocular motility. The affected patients complain of recurrent transient diplopia secondary to a sudden, painless deviation of one eye. This deviation occurs in the direction of action of an extraocular muscle, which is being stimulated at high frequency. ONM is usually seen after radiation therapy, but may also be caused by compression of the affected cranial nerve. The pathophysiology of ONM is incompletely understood, potential mechanisms include 1) ephaptic transmission along the affected nerve, 2) disturbances of potassium channels in the neuronal cell membrane, and 3) central neural re-organisation. The diagnosis of ONM is made based on clinical findings and can be supported by electrophysiological characteristics. Neuroimaging with attention to the affected cranial nerve should be obtained in order to exclude a compressive cause. Therapy of ONM with carbamazepine is usually effective.

Schlüsselwörter

Neuroophthalmologie - Strabologie - Pathophysiologie - Neuromyotonie

Key words

neuroophthalmology - strabism - pathophysiology - neuromyotonia

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