A 78-Year-Old Man Unable to Speak or Swallow

Elena Cecilia Rosca, MD, PhD; Cristina Dijmarescu, MD; Mihaela Simu, MD

Disclosures

January 10, 2018

Discussion

Pseudobulbar paralysis caused by bilateral opercular lesions, or Foix-Chavany-Marie syndrome (FCMS), was diagnosed on the basis of the patient's neurologic signs and neuroimaging findings. The clinical manifestations indicating paralysis of volitional facial, masticatory, pharyngeal, and lingual muscle movements, with preserved movement of these muscles with autonomic functions and emotion, are the defining features of FCMS.

The cortical form of pseudobulbar palsy is distinguished from a noncortical pseudobulbar palsy by the absence of emotional lability or pathological laughter and crying and the absence of bladder and bowel incontinence. Furthermore, brain MRI confirmed the presence of ischemic lesions in both anterior opercula.

FCMS is a rare form of pseudobulbar paralysis caused by bilateral lesions of the anterior opercular cortex. Although it was first described in 1837 by Magnus,[1] FCMS was named after the French authors who investigated it more extensively in 1926.[2,3] A congenital form of the syndrome was reported in 1953 by Worster-Drought.[4]

On the basis of etiology, five forms of FCMS have been proposed[5]:

  • Classical acute form: This is most often related to vascular causes. Most cases are due to bilateral sequential, rarely simultaneous,[6] lesions of the anterior operculum. Some cases can be due to a unilateral opercular lesion. Rarely, an opercular lesion associated with a contralateral subcortical lesion can lead to FCMS.[5,7,8] The most common cause of the acute form is cerebral infarct. Less frequently, it has been described in multiple sclerosis,[9]acute disseminated encephalomyelitis,[10]vasculitis,[11]moyamoya disease,[12] and head trauma.[13]

  • Subacute form: This is due to central nervous system infections, such as herpes simplex encephalitis,[14,15]HIV infection,[16]toxoplasmosis,[17] and tuberculous meningitis.[18]

  • Developmental form: This is mostly due to neuronal migration disorders, such as dysgenesis of the opercular cortex.[5,19] The most common radiologic and pathologic findings have included polymicrogyria, incomplete opercular formation, associated absence of septum pellucidum,[20,21] and bilateral perisylvian ulegyria.[22]

  • Reversible form: This is found in patients with epilepsy. Most cases are in children with benign rolandic epilepsy or hemispheric malformations, who develop a reversible FCMS-like condition in status epilepticus.[5,19,23] However, it has also been reported in some adult patients.[24,25]

  • Chronic, slowly progressive FCMS: This is present in neurodegenerative disorders, such as primary progressive aphasia.[26,27]

The present case is a classical acute form of FCMS due to a cerebral infarction. The main differential diagnosis of pseudobulbar syndrome is bulbar paralysis. In FCMS, contrary to bulbar palsy due to lower motor neuron lesions, the brainstem reflexes (eg, jaw jerk) are preserved or hyperactive and no fasciculations or muscle atrophies are noted. However, the gag reflex is absent, and it is not by itself a discriminating sign. Furthermore, the voluntary-automatic dissociation of bulbar muscle is another cardinal sign of pseudobulbar palsy.

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