Abstract Details

Fiox-Chavany-Marie syndrome (FCMS) is a condition with loss of voluntary movements of cranial nerves and can be seen in bilateral opercular lesions usually caused by ischemic strokes. A peculiar characteristic of this syndrome is the preservation of automatic and emotional muscle activity.

A 72-year-old right-handed male with a history of hypertension, hyperlipidemia, metastatic non-small cell lung cancer, obstructive sleep apnea, and non-ST elevating myocardial infarction on aspirin presented with acute onset anarthria, inability to protrude his tongue, and diminished gag reflex. Involuntary and emotional responses such as smiling, and yawning were preserved. CT angiography of the head and neck showed intra and extracranial stenoses including 60% stenosis of the right internal carotid artery. MRI brain revealed an old right opercular stroke as well as a new left opercular infarct. He was a non-responder to aspirin, and thus he was discharged on aspirin 325 mg and clopidogrel 75 mg for 90 days. Following intense rehabilitation, he exhibited significant improvement in spontaneous language production, word retrieval, and fluency at a follow-up visit 2 months following discharge.

A variety of pathophysiological causes are associated with FCMS including cerebrovascular disease, central nervous system infections, developmental anomalies, epilepsy in children, and neurodegenerative disorders. Among them, cerebrovascular disease appears to be the most common cause, as seen in our case. Most reports show that prognosis in FCMS due to ischemic strokes to bilateral opercula tends to be poor without much recovery. Our case is unique because despite bilateral opercular lesions, the patient exhibited significant improvement in language function, speech production, and fluency, therefore establishing the importance of intensive rehabilitation in cases of FCMS.