Foix-Chavany-Marie Syndrome – Diplegia of the Craniopharyngeal Muscles with Dissociation between Voluntary and Automatic Movements

Introduction: Foix-Chavany-Marie syndrome (FCMS) presents clinically with anarthria, dysphagia and paralysis of the craniopharyngeal muscles with automatic voluntary dissociation. The classical and common form is a bilateral opercular lesion, in most cases associated with strokes. Rare causes are viral encephalitis, neurodegenerative diseases, congenital dysplasias and epilepsy. Case Report: We report the case of a 50-year-old female with two cardioembolic brain infarctions despite an anticoagulation therapy after replacement of the aortic valve in 1987. Three years ago she developed an incomplete bilateral infarction of the posterior cerebral artery territory, one month ago an infarction of the right middle cerebral artery with brachiofacial hemiparesis left and hemineglect to the left side. This time she suddenly became mute. Neurological examination revealed a central facio-pharyngeo-glosso-masticatory diplegia while automatic and emotional movements like laughing, crying, coughing or yawning were still possible without any limitation. Additionally there was an anarthria while listening, comprehension, writing and reading remained intact. MRI showed a new infarction of the thalamic region on the left side. Conclusion: The clinical features of FCMS prove different corticonuclear pathways for voluntary and autonomic motor control of craniopharyngeal muscles. Most common is a bilateral opercular lesion. In this patient we found only on the right side a lesion of the opercular region, on the left side there was a thalamic infarction. This is probably the reason for the almost complete clinical improvement within two months. In general the prognosis described in the literature is very poor.