Abstract
Congenital bilateral perisylvian syndrome (CBPS) is a rare neurological disorder characterised
by pseudobulbar palsy, cognitive deficits and epilepsy associated with bilateral perisylvian
cortical dysplasia on neuroimaging studies. We report a long-term follow-up of a 18-years
girl diagnosed with CBPS according to the typical clinical and magnetic resonance
imaging (MRI) features. The patient showed faciopharyngoglossomasticatory diplegia,
severe dysarthria, ataxia, spastic quadriparesis and severe mental retardation. Brain
MRI evidenced bilateral perisylvian cortical dysplasia. Since early life she suffered
from complex febrile seizures and epilepsy consisting of complex partial attacks with
affective manifestations associated with centro-temporal EEG abnormalities. During
18 years of follow-up she was treated with phenobarbital, carbamazepine, lamotrigine,
gabapentin but did not show any significant clinical improvement. Subsequently, monotherapy
with phenytoin (PHT) was followed by a significant clinical improvement. At age 17,
because of adverse effects, PHT was gradually substituted by topiramate (TPM). Full
control of seizures was obtained at the age of 17 years with TPM. EEG abnormalities
throughout the years have been reduced according to the clinical course. These findings
emphasised the importance of long-term follow-up, suggesting that the prognosis for
epilepsy may not be predicted based on the early response to treatment or on the presence
of structural encephalic abnormalities, as reported in the literature.
Keywords
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Article info
Publication history
Accepted:
February 26,
2004
Received in revised form:
February 25,
2004
Received:
January 29,
2004
Identification
Copyright
© 2004 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
