J Korean Med Sci.  1994 Aug;9(4):335-340. 10.3346/jkms.1994.9.4.335.

Congenital bilateral perisylvian syndrome: analysis of the first four reported Korean patients

• Kim HI
• Palmini A
• Choi HY
• Kim YH
• Lee JC

• KMID: 644817
• DOI: http://doi.org/10.3346/jkms.1994.9.4.335

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.

Keyword

Congenital bilateral perisylvian syndrome; Neuronal migration disorder; Epilepsy; Dysarthria

MeSH Terms

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