Botulinum Toxin Injections for Managing Orofacial Dystonia and Sialorrhea in Foix-Chavany-Marie Syndrome: A Case Report

Case Diagnosis: Foix-Chavany-Marie Syndrome (FCMS) with orofacial dystonia, bruxism, sialorrhea, and anarthria

Case Description or Program Description: A 63-year-old man with a history of ischemic stroke involving the left basal ganglia (BG) and corona radiata (CR) developed persistent sialorrhea, bruxism, orofacial dystonia (OD), anarthria, impaired voluntary facial movements, jaw hypertonicity (JH), and involuntary jaw abduction (IJA) leading to the diagnosis of the rare syndrome FCMS. Anarthria, dystonia, and sialorrhea were likely due to corticobulbar damage, which track courses through the corona radiata, synapsing at cranial nerves (CN) V,VII & IX nuclei. Aphasia, bruxism, OD were likely due to Broca’s damage and disruption of BG circuits involving motor modulation. Communicating primarily via an iPad, he experienced significant difficulty with OD, and persistent sialorrhea which disrupted daily life.
Despite previous trials of scopolamine and glycopyrrolate, symptom relief was inadequate. To address these, he received botulinum toxin injections (total dose: 200 units) into the bilateral masseter, lateral pterygoid, and parotid glands under EMG guidance.

Setting: Outpatient PM&R Clinic

Assessment/Results: After the injections, the patient was scheduled for follow-up to assess treatment response, and his glycopyrrolate dose was increased to better manage sialorrhea. He tolerated the procedure well, with no reported side effects such as excessive weakness or additional swallowing difficulties. Plans were made to continue botulinum toxin therapy as part of his ongoing care.

Discussion (relevance): FCMS is a rare neurological disorder, typically caused by bilateral anterior opercular lesions, but subcortical strokes affecting corticobulbar pathways—such as this patient’s left BG and CR infarct—can cause similar deficits by disrupting bilateral CN control. His OD and sialorrhea were effectively managed with botulinum toxin injections, improving function and quality of life.

Conclusions: Botulinum toxin therapy presents a viable approach for treating neuromuscular dysfunction in FCMS. With limited treatment options available, this method can provide meaningful symptom relief and improve functional outcomes in affected individuals.