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  • Title: Opercular syndrome without opercular lesions: Foix-Chavany-Marie syndrome in progressive supranuclear motor system degeneration.
    Author: Weller M, Poremba M, Dichgans J.
    Journal: Eur Arch Psychiatry Neurol Sci; 1990; 239(6):370-2. PubMed ID: 2144237.
    Abstract:
    A patient is described with slowly progressive supranuclear motor system degeneration (primary lateral sclerosis) characterized by pure bulbar spasticity for six years until a spastic tetraparesis developed. Clinically and electrophysiologically there was and still is no evidence of lower motor neuron involvement. Recently the patient presented with a syndrome of complete inability to move face and tongue voluntarily with preservation of the ability to move them "automatically", e.g. within gestures: automatic-voluntary motor dissociation. Loss of voluntary innervation of the facio-pharyngo-glossomasticatory muscles with preservation of involuntary innervation are the features of the Foix-Chavany-Marie syndrome, which to date has most often been described in association with bilateral vascular lesions of the opercula or their cortigofugal projections.
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