Congenital bilateral perisylvian syndrome: analysis of the first four reported Korean patients.
10.3346/jkms.1994.9.4.335
- Author:
Hyoung Ihl KIM
1
;
Andre PALMINI
;
Ha Young CHOI
;
Yun Hee KIM
;
Jung Chung LEE
Author Information
1. Department of Neurosurgery, Chonbuk National University Medical School, Chonju, Republic of Korea.
- Publication Type:Original Article ; Clinical Trial
- Keywords:
Congenital bilateral perisylvian syndrome;
Neuronal migration disorder;
Epilepsy;
Dysarthria
- MeSH:
Adolescent;
Adult;
Anticonvulsants/therapeutic use;
Cerebral Cortex/*abnormalities;
Dysarthria/*diagnosis/therapy;
Electroencephalography;
Epilepsy, Generalized/congenital/*diagnosis/therapy;
Evoked Potentials, Somatosensory;
Facial Paralysis/congenital/*diagnosis/therapy;
Female;
Follow-Up Studies;
Human;
Magnetic Resonance Imaging;
Male;
Mental Retardation/*diagnosis/therapy;
Surgical Procedures, Operative/methods;
Syndrome
- From:Journal of Korean Medical Science
1994;9(4):335-340
- CountryRepublic of Korea
- Language:English
-
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.
