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Advances in neuroimaging techniques have enabled the recog-nition of developmental malformations of the brain during life.Careful correlation of clinical and imaging features has identified several new syndromes.We have studied 31 patients with a congenital neurological syndrome characterised by pseudobulbar palsy,cognitive deficits,and bilateral perisylvian abnormalities on the imagine studies.All patients had diplegia of the facial pharyngeal,and masticatory muscles,of variable severity.Some patients had slight dysarthria,whereas others were unable to speak.85%of patients with mental retardation,ranging from mild to severe.Epilepsy was present in 27(87%)and commonly consisted of atypical absence,atonic/tonic, tonic-clonic seizures,and,less frequently,partial attacks.Seizures were poorly controlled in 55%.Magnetic resonance imaging showed bilateral perisylvian cortical malformations consistent with polymicrogyria. confirmed at necropsy.Divisions of the corpus callosum in several patients resulted in seizure improvement.The congenital bilateral perisylvian syndrome can be clinically diagnosed and confirmed by imaging studies. Further studies are necessary to elucidate its cause. |
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clubfoot as related to neurologic disease
congenital bilateral perisylvian syndrome
congenital malformation
developmental abnormality of brain
developmental retardation
drooling
dysarthria
dyskinesia,buccal lingual facial
dysphagia
electroencephalogram,abnormalities of
facial weakness,bilateral
gag reflex,depressed
intellectual deficit
malformation,CNS,congenital
mental retardation
MRI
MRI,abnormal
nonverbal
operculum syndrome,bilateral
polymicrogyria
pseudobulbar palsy
pyramidal tract dysfunction
seizure
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