CLINICAL SPECTRUM OF FOIX CHAVANY MARIE SYNDROME - AN ANALYSIS OF FIVE CASES

Foix-Chavany-Marie syndrome (FCMS), is characterized by a loss of voluntary control of facial, lingual, pharyngeal and masticatory muscles, in the presence of preserved reflexive and automatic functions (Automatic Voluntary Movement Dissociation) of the same muscles. Also known as Opercular Syndrome , FacioLabio-Pharyngo-Glosso-Masticatory Paralysis has varied aetiology and presentation with Vascular causes being chief among them.We present here five cases of FCMS admitted in our institute with etiological and clinical spectrum analysis. MATERIALS AND METHODS Five patients of FCMS provisionaly diagnosed based on clinical features of cortical pseudobulbar palsy features admitted in our Madras Istitute Of Neurology between 2008 -2011 were analysed on history, clinical forms and imaging with CT or MRI. Routine investigations were done to screen for etiological factors. DISCUSSION All the patients had classical features of facio-labiopharyngoglosso-masticatory paralysis with automatic -voluntary dissociation as evident by preserved movements of facial muscles and opening of mouth during yawning, coughing and smiling. Among five patients three had their onset before 40 years. Stroke was the etiology in four of the patients and in one patient Takayasus arteritis was diagnosis which has been rarely reported and one case presented after encephalitis. Based on site of lesion three were bilateral Anterior Opercular, two were unilateral Anterior Syndrome Involving Frontal Operculum. CONCLUSION FCMS is a syndrome which is less recognised by many physicians.These patients invariably land up in gastroenterolgy department for dysphagia. Early.diagnosis of patients with CT,MRI, SPECT is a must for appropriate assessment and therapeutic approaches. Takayasu's Arteritis presenting as FCMS is an unusual etiology. Apart from vascular cause, HSV encephalitis, seizures, neuronal migration disorders also present as FCMS