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.