J Korean Child Neurol Soc.  1998 May;5(2):292-302.

Bilateral Cortical Dysplasias : MRI Findings and Clinical Aspects

• Kim EY
• Kim GM
• Woo YJ

Abstract

PURPOSE: Cerebral cortical dysplasias are one of the important causes for epileptic seizures and developmental disabilities in children, particularly in diffuse or bilateral cases. These developmental malformations are generally regarded as a group of neuronal migration disorders, however, the classification system and pathogenetic mechanisms of cortical dysplasias are not yet entirely clear. Even a novel entity, congenital bilateral perisylvian syndrome, characterized by speech delay, pseudobulbar Palsy, intractable seizures, and bilateral perisylvian abnormalities on imaging studies, have rather diverse figures on morphology and symptomatology than initially considered. We have studied the clinical features and correlations of clinical outcomes 3nd magnetic resonance imaging(MRI) findings of bilaterally involved cortical dysplasias.
METHODS
20 cases of bilateral cortical dyspalsias were grouped into three categories on the basis of MRI findings; centroparietal dysplasias(6 cases), diffuse dysplasias with (5) or without white matter lesions(4), and schizencephaly(5). EEGs, motor and language development, epilepsy, and outcomes were reviewed in each groups.
RESULTS
Language delay(100%), motor developmental delay(94.7%), motor deficit(65%), epilepsy(40%) were the main reasons for their initial hospital visits. A meaningful word expression was possible at the mean age of 2 years and 2 months(1 to 4 years of age). Hypotonia and spastic motor paralysis were evident in all the cases of diffuse dysplasia with white matter lesions and the schizencephaly groups. All but one case of centroparietal dysplasia showed motor developmental delay. Epilepsies were developed in 8 cases at the mean age of 5 years and 5 months(2 months to 12 years of age) and the seizures were relatively well controlled with anticonvulsants. EEG findings were variable; normal, focal or diffuse abnormalities. High amplitude diffuse fast activities were only noted in the diffuse dysplasia group.
CONCLUSION
The severity of neurological deficits and developmental delay had correlated to the size of cortical abnormalities. The epilepsies were relatively well controlled during childhood and the EEG finding of high amplitude diffuse fast activities was specific for the diffuse cortical dysplasias. Bilateral centroparietal dysplasias should be included on consideration of the causes for developmental aphasia.

MeSH Terms

Anticonvulsants
Aphasia
Child
Classification
Developmental Disabilities
Electroencephalography
Epilepsy
Humans
Language Development
Language Development Disorders
Magnetic Resonance Imaging*
Malformations of Cortical Development*
Muscle Hypotonia
Muscle Spasticity
Neuronal Migration Disorders
Paralysis
Pseudobulbar Palsy
Seizures
Anticonvulsants