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Foix–Chavany–Marie syndrome (FCMS) is characterised clinically by automatic voluntary dissociation of orofacial motility. It is caused by bilateral anterior opercular lesions and its aetiology is heterogeneous.1
Clinically, most cases of FCMS can be divided into three categories—developmental, acute/subacute, and transient.2 The most common cause of the developmental form is congenital bilateral anterior opercular dysplasia. The acute/subacute form is usually caused by infection in the CNS or cerebrovascular disease. The underlying pathogenesis of transient form is epilepsy. Rare variant cases of FCMS presenting with a slowly progressive clinical course have also been reported.3 We describe the clinical features of a patient with adult onset, slowly progressive FCMS, thought to be associated with chronic herpes simplex encephalitis.
A 29 year old Japanese woman developed epilepsy of generalised tonic-clonic type at the age of 15 and had been taking anticonvulsants for 14 years. She had had a normal pregnancy and delivery. Developmental assessment during schooling showed normal motor and psychological ability.
At the age of 27, she presented with dysarthria and dysphagia, which deteriorated gradually during the following 18 months. She also had difficulty with fine movements of her right arm at the age of 29. On admission, her voluntary orofacial motility was disturbed bilaterally, while emotional and involuntary facial movements …