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Ophthalmoplegia plus, an electro-oculographic study

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Abstract

To find out whether the central structures governing conjugate eye movements are affected in ophthalmoplegia plus (OP), we conducted an electro-oculographic study in 6 patients suffering from OP with varying degrees of extraocular involvement and with mitochondrial changes in the skeletal musculature. All the patients presented alterations of the smooth pursuit and saccadic movements, of optokinetic nystagmus as well as of the vestibular reflexes and of visuovestibular interaction, thus confirming impairment of the central oculomotor structures and particularly of those of the archicerebellum and brainstem. These data suggest a supranuclear component in the pathogenesis of ophthalmoplegia and are in accord with the findings of brainstem spongiosis in OP, which in turn seem to express a multisystem pathological state of the mitochondria.

Sommario

Allo scopo di accertare nell'Oftalmoplegia plus (O.P.) l'eventuale interessamento delle strutture centrali deputate al controllo della motilità oculare coniugata è stato eseguito uno studio elettro-oculografico in 6 pazienti affetti da oftalmoplegia progressiva con diverso grado di interessamento extraoculare e con alterazioni mitocondriali della muscolatura scheletrica. Tutti i pazienti hanno mostrato alterazioni dei movimenti oculari di smooth-pursuit e saccadici, del nistagmo optocinetico, nonché dei riflessi vestibolari e dell'interazione visuo-vestibolare, confermando così una compromissione delle strutture oculomotrici centrali ed in particolare di quelle archicerebellari e del tronco encefalico. Questi dati suggeriscono una componente sovranucleare nella patogenesi dell'oftalmoplegia e sono in accordo con i reperti di spongiosi del tronco encefalico riscontrati nei pazienti con OP che a loro volta appaiono espressione di una patologia mitocondriale plurisistemica.

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Authors and Affiliations

  1. Istituto delle Malattie Nervose e Mentali della Università di Firenze, Firenze, Italia

    Marconi G.

  2. Servizio di Audiologia, Università di Firenze, Firenze, Italia

    Pagnini P. & Vannucchi P.

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  1. Marconi G.
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Marconi, G., Pagnini, P. & Vannucchi, P. Ophthalmoplegia plus, an electro-oculographic study. Ital J Neuro Sci 6, 429–439 (1985). https://doi.org/10.1007/BF02331035

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