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The nosological position of Fisher's syndrome (Ophthalmoplegia, Ataxia and Areflexia)

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Summary

Fisher's syndrome was studied in six patients, one of whom was an 11-year-old girl, and the nosological position of this neuro-opthalmological disorder was reviewed. The clinical features of the ophthalmoplegia in these cases were reported as secondary to a transient inflammatory lesion in the mid-brain; this was demonstrated in one of the cases by an enhancing area on CT scan. This mid-brain localization could explain not only the classical Fisher's triad but also many variations of the syndrome. The overlap of our cases of Fisher's syndrome with so-called brain-stem encephalitis of Bickerstaff has been emphasized. Many cases of Fisher's syndrome could therefore correspond to an inflammatory process at brain-stem level with or without nerve-root involvement. Clinical and neurophysiological findings show, however, that there is peripheral damage in some cases, so that a neutral attitude needs to be maintained on the nosological position of Fisher's syndrome.

Zusammenfassung

Es werden sechs Fälle von Fisher-Syndrom beschrieben, wovon einer ein elfjähriges Mädchen war, und es wird die nosologische Stellung des Syndromes diskutiert. Aufgrund der klinischen Besonderheiten der Augenmotilitätsstörungen in den vorliegenden Fällen nahmen die Autoren als Ursache eine vorübergehende entzündliche Läsion des Mittelhirnes an. Dies wurde in einem Fall durch das Vorhandensein einer Zone mit vermehrter Dichte im Computertomogramm belegt. Eine Lokalisation im Mittelhirn könnte nicht nur die drei klassischen Hauptsymptome des Fisher-Syndroms erklären, sondern auch zahlreiche seiner Varianten. Es wird die Überlappung der eigenen Beobachtungen von Fisher-Syndrome mit der sogenannten Hirnstammencephalitis von Bickerstaff hervorgehoben. Manche Fälle von Fisher-Syndrom könnten somit einem entzündlichen Prozeß des Hirnstammes mit oder ohne gleichzeitigem Betroffensein von Nervenwurzeln entsprechen. Klinische und neurophysiologische Untersuchungen allerdings zeigen, daß in manchen Fällen auch eine periphere Schädigung vorliegt, so daß manche Aspekte in bezug auf die nosologische Stellung des Syndromes noch offengelassen werden müssen.

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Barontini, F., Sitá, D. The nosological position of Fisher's syndrome (Ophthalmoplegia, Ataxia and Areflexia). J Neurol 229, 33–44 (1983). https://doi.org/10.1007/BF00313494

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