Pure congenital Foix-Chavany-Marie syndrome.
Foix-Chavany-Marie syndrome (FCMS) is characterized by facio-linguo-masticatory diplegia in the absence of limb weakness. The most common cause is a cortical lesion resulting from a stroke but a congenital form has been reported. We present the case of a 53-year-old man who was admitted to hospital with worsening dysphagia which was know to have been present together with anarthria and facial palsy, since birth. He demonstrated features of FCMS with pseudobulbar palsy and unaffected reflexes and automatic responses. Cranial CT and MRI scans showed bilateral opercular lesions of CSF intensity in continuity with the lateral ventricles. We conclude that this case of static FCMS for over 50 years may represent a 'pure' form of congenital FCMS with motor symptomatology and unaccompanied by mental retardation or epilepsy.
Nisipeanu
P
P
Sacker Faculty of Medicine, Department of Physiology and Pharmacology, Tel Aviv University, Ramat Aviv, Israel.
Rieder
I
I
Blumen
S
S
Korczyn
A D
AD
D001927:Brain Diseases / Q000151:congenital* / Q000175:diagnosis; D002294:Carcinoma, Squamous Cell / Q000150:complications / Q000175:diagnosis; D002552:Cerebral Ventricles / Q000002:abnormalities; D003680:Deglutition Disorders / Q000209:etiology*; D004938:Esophageal Neoplasms / Q000150:complications / Q000175:diagnosis; D005158:Facial Paralysis / Q000209:etiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D013577:Syndrome; D014057:Tomography, X-Ray Computed