Summary
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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Weller, M., Poremba, M. & Dichgans, J. Opercular syndrome without opercular lesions: Foix-Chavany-Marie syndrome in progressive supranuclear motor system degeneration. Eur Arch Psychiatr Neurol Sci 239, 370–372 (1990). https://doi.org/10.1007/BF01734544
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DOI: https://doi.org/10.1007/BF01734544