Progressive Anterior Operculum Syndrome

—Correlation to Apraxia of Speech with Respect to Semiology and Etiology—

Abstract

Foix-Chavany-Marie syndrome, or anterior operculum syndrome, is a cortical type of pseudobulbar palsy characterized by anarthria and facio-linguo-pharyngo-laryngo-glosso-masticatory diplegia with “automatic voluntary dissociation”. Besides the classical and most common form associated with cerebrovascular disease, the syndrome may be caused by central nervous system infections, neuronal migration disorders, epilepsy, or neurodegenerative disorders. The neurodegenerative form of the syndrome should be considered a variant of frontotemporal lobar degeneration. The semiology, topography of the responsible lesion, and etiology of the progressive anterior operculum syndrome are discussed and compared to apraxia of speech.