Opercular syndrome without opercular lesions: Foix-Chavany-Marie syndrome in progressive supranuclear motor system degeneration.
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.
Weller
M
M
Department of Neurology, University Clinic, Tübingen, Federal Republic of Germany.
Poremba
M
M
Dichgans
J
J
D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis*; D002540:Cerebral Cortex / Q000473:pathology*; D003937:Diagnosis, Differential; D004401:Dysarthria / Q000175:diagnosis; D015417:Hereditary Sensory and Motor Neuropathy / Q000175:diagnosis*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009132:Muscles / Q000294:innervation; D009410:Nerve Degeneration / Q000502:physiology; D009460:Neurologic Examination; D010243:Paralysis / Q000175:diagnosis*; D015419:Spastic Paraplegia, Hereditary / Q000175:diagnosis*; D013577:Syndrome