Rapid Progressive Dysphagia in Foix-Chavany-Marie Syndrome with Lennox-Gastaut Syndrome
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Foix-Chavany-Marie syndrome (FCMS), or bilateral anterior opercular syndrome, is a rare form of pseudobulbar palsy characterized by an automatic-voluntary dissociation of the facio-pharyngo-glosso-masticatory muscles. Although dysphagia is a major clinical feature of FCMS, few studies have described its longitudinal progression, particularly in the context of epileptic encephalopathy. This paper reports a 32-year-old male with long-standing Lennox-Gastaut syndrome secondary to childhood herpes simplex encephalitis who developed FCMS with rapidly progressive dysphagia. Brain magnetic resonance imaging revealed multifocal old infarcts involving the bilateral frontoparietal opercular regions on a background of chronic encephalomalacia. Clinically, the patient exhibited classic automatic-voluntary dissociation. Serial videofluoroscopic swallowing studies (VFSS) over two months revealed rapid deterioration, from selective penetration with preserved semisolid swallowing to universal silent aspiration across all consistencies. Unlike typical FCMS caused by acute bilateral ischemic stroke, this case suggests a cumulative, epileptic network-mediated mechanism. The VFSS findings support a functional decompensation model, in which exhaustion of the swallowing network reserve may lead to abrupt functional collapse without radiologic evidence of a new structural lesion. Serial VFSS provides critical functional insight into the disease progression in FCMS and may guide appropriate decisions regarding long-term enteral nutrition.
Abstract
Foix-Chavany-Marie syndrome (FCMS), or bilateral anterior opercular syndrome, is a rare form of pseudobulbar palsy characterized by an automatic-voluntary dissociation of the facio-pharyngo-glosso-masticatory muscles. Although dysphagia is a major clinical feature of FCMS, few studies have described its longitudinal progression, particularly in the context of epileptic encephalopathy. This paper reports a 32-year-old male with long-standing Lennox-Gastaut syndrome secondary to childhood herpes simplex encephalitis who developed FCMS with rapidly progressive dysphagia. Brain magnetic resonance imaging revealed multifocal old infarcts involving the bilateral frontoparietal opercular regions on a background of chronic encephalomalacia. Clinically, the patient exhibited classic automatic-voluntary dissociation. Serial videofluoroscopic swallowing studies (VFSS) over two months revealed rapid deterioration, from selective penetration with preserved semisolid swallowing to universal silent aspiration across all consistencies. Unlike typical FCMS caused by acute bilateral ischemic stroke, this case suggests a cumulative, epileptic network-mediated mechanism. The VFSS findings support a functional decompensation model, in which exhaustion of the swallowing network reserve may lead to abrupt functional collapse without radiologic evidence of a new structural lesion. Serial VFSS provides critical functional insight into the disease progression in FCMS and may guide appropriate decisions regarding long-term enteral nutrition.