Foix-Chavany-Marie Syndrome Case Series
Neeharika Krothapalli1, Vladislav Zayas2, Isaac Silverman3
1Neurology, University of Connecticut, 2Neurology, Hartford Hospital, 3Hartford Hospital
Objective:

To report two patients with Foix-Chavany-Marie Syndrome (FCMS) and highlight the importance of early recognition.

Background:

FCMS is a rare type of cortical-subcortical bulbar paralysis characterized by automatic-voluntary dissociation of facio-labio-pharyngo-masticatory movements. Most cases occur due to bilateral ischemic lesions of the anterior operculum but there are also cases with unilateral opercular damage. Clinical manifestations include severe dysarthria or anarthria, aphasia, generalized facial weakness, loss of voluntary muscular functions of face and tongue, difficulty with mastication or swallowing, seizures, and preservation of reflex or automatic movements. Here, we report two individuals at our institution who were diagnosed with FCMS, which has rarely been reported in a case series.

Design/Methods:

We included two patients diagnosed with FCMS. Data was gathered from chart review.

Results:

Case 1: A 93-year-old woman with prior left hemispheric stroke presented with speech difficulty, difficulty opening her mouth, and drooling. Examination was notable for anarthria and difficulty with labial movements. She performed better with producing labial rather than lingual or guttural sounds. MRI revealed an acute ischemic infarct in the right opercular region with a chronic infarct in the left opercular area. Case 2: A 75-year-old woman with prior left opercular ischemic stroke and atrial fibrillation on Apixaban was admitted for partial nephrectomy for renal mass. During her hospitalization, she developed acute onset facial and bulbar weakness with anarthria and dysphagia. MRI confirmed a new ischemic infarct in the right middle cerebral artery territory involving the operculum. Etiology was cardioembolic in the setting of anticoagulation held for surgery. Both patients underwent early evaluation by speech therapy to guide rehabilitation in the acute setting.

Conclusions:

Foix-Chavany-Marie Syndrome is characterized by facio-labio-pharyngo-glosso-masticatory paralysis. There is automatic-voluntary dissociation with preservation of involuntary movements. Although rare, early recognition of this entity enables anticipation of challenges to respiratory and nutritional management.

10.1212/WNL.0000000000204708