Clinical analysis of Foix-Chavany-Marie syndrome

Abstract

Objective To summarize the clinical characteristics of Foix-Chavany-Marie syndrome. Methods The clinical, electroencephalography and imaging characteristics, as well as etiology, treatment and prognosis of 4 cases of Foix-Chavany-Marie syndrome were retrospectively evaluated, and relevant literature was reviewed to investigate its pathogenesis. Results All the 4 cases presented with hypersalivation, facial diplegia, dysarthria and dysphagia with autonomic-voluntary dissociation. Brain MRI showed bilateral cortical or subcortical lesions in the opercula and perisylvian areas. Three cases were secondary to encephalitis, and 1 case was related to perinatal hypoxic-ischemic encephalopathy. After administration of antiepileptic drugs, they were free of seizures. But all the other symptoms remained. Conclusions Autonomic-voluntary dissociation is a feature of Foix-Chavany-Marie syndrome. Most patients are due to bilateral structural or functional lesions between motor cortices and brain-stem cranial motor nuclei. The prognosis depends on the heterogeneous etiologies, such as encephalitis and cerebrovascular disease. Key words: Foix-Chavany-Marie syndrome; Cortical pseudobulbar palsy; Pathogenesis