Congenital bilateral perisylvian syndrome: analysis of the first four reported Korean patients
Journal of Korean Medical Science
;
: 335-340, 1994.
Article
in English
| WPRIM
| ID: wpr-162665
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Surgical Procedures, Operative
/
Syndrome
/
Magnetic Resonance Imaging
/
Cerebral Cortex
/
Follow-Up Studies
/
Epilepsy, Generalized
/
Dysarthria
/
Electroencephalography
/
Evoked Potentials, Somatosensory
/
Facial Paralysis
Type of study:
Observational study
/
Prognostic study
Limits:
Adolescent
/
Adult
/
Female
/
Humans
/
Male
Language:
English
Journal:
Journal of Korean Medical Science
Year:
1994
Type:
Article
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