PEDIATRIC CONGENITAL BILATERAL PERISYLVIAN SYNDROME - CLINICAL AND MRI FEATURES IN 12 PATIENTS

In 1926, Foix, Chovany and Marie described an acquired syndrome of fas ciopharyngoglossomasticatory diplegia resulting from bilateral infarct ion of the anterior operculum. Clinical features consisted of facial d iplegia, dysarthria, pseudobulbar palsy, mild to severe mental retarda tion, and seizures, A developmental form, similar in presentation in a dults with MRI findings consisting of bilateral perisylvian cortical m alformation consistent with polymicrogyria involving the sylvian fissu re and opercular cortex, has been recognized; but few pediatric cases of congenital bilateral perisylvian syndrome (CBPS) have been reported , Over the past four years, we have encountered 12 cases of CBPS prese nting in childhood. Age ranges were from 1 week to 11 years with a med ian of 2.25 years; six were less than two years of age. Seven were mal e and five female. Ten had bilateral perisylvian polymicrogyria on MRI ; two had unilateral perisylvian schizencephaly with contralateral per isylvian polymicrogyria. Clinical manifestations included developmenta l delay in 7; poor palatal function in 5; hypotonia in 4; arthrogrypos is in 4; hemiparesis in 3; apnea in 3; paraparesis in 2; micrognathia in 2; pectus excavatum in 2; quadriparesis in 1; and hearing loss in 1 . Seizures occurred in seven (58%) and consisted of infantile spasms ( n = 1), generalized tonic-clonic (n = 1), complex partial (n = 2), par tial motor (n = 2; 1 with secondary generalization), and febrile convu lsions (n = 1), CBPS has different manifestations in the pediatric pop ulation than in adults. CBPS is more common than previously thought, i s recognizable by MRI and should be suspected clinically in any infant or child presenting with oromotor dysfunction/pseudobulbar signs and developmental delay, especially if there are associated congenital mal formations. Epilepsy is not a constant feature in the pediatric presen tation and is variable in type and severity.