ABSTRACT
We report a young man with congenital bilateral perisylvian syndrome (CBPS), who showed eating reflex seizures (ERS). His ERS were related to the taste of foods. The subtracted ictal SPECT co-registered to MRI (SISCOM) revealed the hyperperfusion in the insula as well as the subcortical nuclei and brainstem. The ERS in CBPS may be partially related with the taste function of the insula and SISCOM may be helpful for the localization of the epileptogenic foci in ERS.
Subject(s)
Humans , Brain Stem , Eating , Magnetic Resonance Imaging , Reflex , Seizures , Tomography, Emission-Computed, Single-PhotonABSTRACT
Congenital bilateral perisylvian syndrome(CBPS) is rare in literature,especially in China.In this article,we report the clinical and treatment of a patient with CBPS and discuss its mechanism,clinical features and therapy.This patient was a 28-year-old man.His main clinical features were pseudobulbar palsy,cognitive deficits and intractable epilepsy.MRI shows bilateral thickening of the cortex around the sylvian fissures which were deeper than normal and polymicrogyria.The electroencephalogram demonstrated slow spike in right temporal lobe and left frontal lobe.Rhythmal 4 Hz ? waves exist in left frontal and parietal lobe.As the epilepsy was poorly controlled by antiepileptic,section of the corpus callosum was carried out.After callosotomy,there was pronounced seizure reduction and intelligence development improvement.CBPS is characterized by pseudobulbar palsy,cognitive deficits,and bilateral perisylvian abnormalities in imaging studies.If intractable epilepsy is combined,callosotomy may be effective.
ABSTRACT
The advent of MRI technique has enabled the diagnosis of neuronal migration disorders(NMD) and made it possible to make "in vivo" diagnosis. Congenital bilateral perisylvian syndrome(CBPS) is a recently described disease identify characterized by pseudobulbar palsy, epilepsy, mental retardation, and migration disorders in the bilateral perisylvian area. We have identified four CBPS patients based on neuroimaging and dysarthria patterns among the candidates for epilepsy surgery. All the patients had orofacial diplegia and variable degrees of mental retardation. In the spectrographic analysis of dysarthria, the loss of specific characteristics of formants of vowels and increment of noise in the high frequency formants were observed. Epilepsy was present in all, but only one patient showed intractable seizure requiring surgical intervention. MRI was most helpful in identifying NMD and polymicrogyria in both centroparietal areas in this context. Great alertness is needed to identify this disorder to determine the etiology of epilepsy and dysarthria of uncertain origin.