Related Experiment Videos
Congenital unilateral perisylvian syndrome: radiological basis and clinical correlations
1Département de Pédiatric, Hôpital Bicetre, Le Kremlin Bicetre, France.
Insights
This study identifies a new brain malformation syndrome in children, characterized by specific imaging findings and developmental impairments. The condition appears to occur sporadically, offering insights into prognosis and clinical management.
Area of Science:
- Neuroimaging
- Developmental Neuroscience
- Clinical Neurology
Background:
- Neuroimaging advances enable correlation of radiological patterns with clinical features of brain malformations.
- A novel neuroimaging pattern involving unilateral sylvian fossa widening and abnormal perisylvian cortex is described.
Purpose of the Study:
- To report the clinical, prognostic, and electroencephalographic (EEG) features of six children with this previously unrecognized neuroimaging picture.
- To validate a unilateral perisylvian syndrome.
Main Methods:
- Case series of six children with the described neuroimaging findings.
- Clinical assessment, neuroimaging review, and electroencephalography (EEG).
Main Results:
- Children presented with reduced ipsilateral hemisphere size, thalamostriatal hypoplasia, and hemiplegia.
- Cognitive development was predominantly impaired; epilepsy occurred in two patients with partial seizures.
- EEG revealed hemispheric slowing of background activity contralateral to the perisylvian dysplasia; no familial occurrence was noted.
Conclusions:
- The findings confirm a distinct clinical picture, sporadic occurrence, and prognosis for this brain malformation.
- The study validates the recognition of a unilateral perisylvian syndrome.
Design:
Advances in neuroimaging have allowed correlations between radiological patterns and clinical features of brain malformations. This paper reports clinical, prognosis, and electroencephalographic features of six children with a previously unrecognised neuroimaging picture of unilateral widening and verticalisation of the sylvian fossa associated with an abnormal ipsilateral perisylvian cortex.
Results:
All children had reduced hemisphere size and thalamostriatal hypoplasia ipsilateral to the cleft and hemiplegia. Cognitive development was mostly impaired. Epilepsy occurred in two patients and was mainly characterised by partial seizures. Studies with EEG showed hemispheric slowing of background activity homolateral to the perisylvian dysplasia. Occurrence of the malformation among their siblings was not found.
Conclusion:
Similar brain malformations occasionally reported in older patients confirm the clinical picture, sporadic occurrence, and prognosis found, allowing the validation of a unilateral perisylvian syndrome.