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

Abstract

INTRODUCTION
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 , Facio-
Labio-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