Reversible opercular syndrome secondary to osmotic demyelination

Challenging Cases in Neurology: Neurologic Complications of COVID-19

“…MRI in patients with ODS may also show T2/FLAIR hyperintensities with associated T1 hypointensity involving pontine and extrapontine sites (Figure 5) or, in the acute setting, areas of restricted diffusion. 12 His prior unilateral opercular injury may have predisposed to FCMS in the setting of a second, bilateral insult in the form of ODS.…”

“…The patient's neurologic examination was most consistent with an anterior biopercular syndrome, eponymously termed “Foix-Chavany-Marie syndrome (FCMS).” FCMS consists of autonomic-voluntary dissociation of orofacial motility, most often caused by bilateral anterior perisylvian lesions involving the primary motor cortex and parietal opercula, although it has rarely been described in patients with unilateral opercular lesions, either in isolation or associated with a lesion of the contralateral cortico-nuclear tract. 12 13 FCMS is characterized by significant dysphagia and apraxia of the muscles of the mouth with inability to execute familiar motor routine activities such as speaking and swallowing. Reflexive and automatic functions of these muscles, including laughter and yawning, are usually preserved.…”

“…17 Rarely, FCMS has been described in patients with osmotic demyelination syndrome (ODS). 12,18 Although ODS is most often seen after rapid correction of chronic hyponatremia, it has also been described in association with other diseases that cause fluid and electrolyte disturbances. Patients with renal failure, including this patient, may be predisposed to ODS by their renal disease or by osmotic changes during hemodialysis.…”

A Case of Foix-Chavany-Marie Syndrome With Asynchronous Bilateral Opercular Infarcts and Chronic Bilateral Cerebellar Infarcts

“…This syndrome was first described by Magnus in 1837 and further defined by Foix, Chavany, and Marie in 1926 after whom the condition was later named [ 4 ]. FCMS is commonly caused by ischemic lesions [ 1 - 4 ]; however, it has also been reported in patients with cerebral infection [ 5 - 7 ], demyelinating disease [ 8 ], trauma [ 9 ], tumor [ 10 ], hydrocephalus [ 11 ], and osmotic demyelination syndrome [ 12 ]. There have been less than 150 cases of FCMS noted in the literature, and less than 40 cases of which have been caused by ischemic stroke; however, FCMS is being increasingly reported due to advances in computed tomography (CT) and magnetic resonance imaging (MRI) [ 3 , 4 ].…”

Importance of Rapid Clinical Recognition of the Anterior Opercular Syndrome (Foix-Chavany-Marie Syndrome): A Case Report

“…The first case was reported by Magnus in 1837 [2], who described a patient with mutism and bilateral loss of voluntary movement of the musculature innervated by the cranial nerves V, VII, IX, X, and XII, with preserved reflexes, automatic and emotional functions referred to as automatic-voluntary dissociation [2]. Affected individuals typically have anarthria and dysphagia, with preserved ability to close their eyes during sleep as well as involuntarily yawn, smile, laugh, and cry [1, 2], AOS has been reported in patients suffering from neuroinfection [3], neuroinflammatory or neurodegenerative diseases [4, 5], brain tumors [6], traumatic brain injury [7], normal pressure hydrocephalus [8], osmotic demyelination [9], but mostly due to acute stroke [10]. It is therefore extremely important to recognize this syndrome as it can have consequences for the further acute treatment, including early revascularization therapy.…”