Premium
Pure congenital Foix‐Chavany‐Marie syndrome
Author(s) -
Nisipeanu P.,
Rieder I.,
Blamen S.,
Korczyn Amos D.
Publication year - 1997
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/j.1469-8749.1997.tb07366.x
Subject(s) - pseudobulbar palsy , diplegia , medicine , palsy , dysphagia , pediatrics , weakness , cerebral palsy , physical medicine and rehabilitation , anatomy , surgery , pathology , alternative medicine
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 SO years may represent a ‘pure’ form of congenital FCMS with motor symptomatology and unaccompanied by mental retardation or epilepsy.