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Congenital perisylvian syndrome: MRI and glucose PET correlations
Aimee F Luat1, Bruno Bernardi, Harry T Chugani
1The Carman and Ann Adams Department of Pediatrics, Childrens Hospital of Michigan, Wayne State University, Detroit, Michigan 48201, USA.
Insights
Congenital perisylvian syndromes involve brain malformations affecting cortical organization. Positron emission tomography (PET) scans reveal functional abnormalities beyond MRI-visible lesions, aiding in assessing clinical severity.
Area of Science:
- Neuroscience
- Developmental Neurology
- Medical Imaging
Background:
- Congenital perisylvian syndromes are neurodevelopmental disorders characterized by abnormal cortical organization.
- These syndromes present with diverse neurological impairments and distinct imaging findings.
Purpose of the Study:
- To evaluate the clinical, magnetic resonance imaging (MRI), and 2-deoxy-2-[18F] fluoro-D-glucose (FDG) positron emission tomography (PET) findings in children with congenital perisylvian syndrome.
- To determine the utility of PET scans in assessing the functional extent of cortical anomalies.
Main Methods:
- Retrospective analysis of clinical data, MRI, and FDG-PET scans from six pediatric patients diagnosed with congenital perisylvian syndrome.
- Correlation of imaging findings with neurological impairments.
Main Results:
- MRI revealed characteristic perisylvian malformations, including vertically oriented sylvian fissures and polymicrogyria, in all patients.
- FDG-PET scans demonstrated metabolic abnormalities in perisylvian regions corresponding to MRI findings, and also in areas with normal MRI.
- Patients with unilateral MRI abnormalities showed contralateral metabolic dysfunction; bilateral malformations correlated with more extensive PET abnormalities.
Conclusions:
- While MRI is crucial for diagnosing the structural lesions of congenital perisylvian syndrome, FDG-PET provides essential functional information.
- PET imaging helps delineate the complete functional impact of cortical anomalies, contributing to a more accurate assessment of clinical severity.
Abstract:
Congenital perisylvian syndromes are late migration/cortical organization disorders associated with distinctive clinical and imaging features. The clinical, magnetic resonance imaging, and 2-deoxy-2-[18F] fluoro-D-glucose (FDG) positron emission tomography scan findings of six children (age range: 3.2-16.7 years; 5 males) with congenital perisylvian syndrome were evaluated. The patients presented with heterogenous neurologic impairments, depending upon the involved hemisphere and the extension of perisylvian malformation. Two manifested bilateral malformation and four manifested unilateral. The characteristic MRI finding consisting of a vertically oriented sylvian fissure continuous with the central and postcentral sulcus was associated with variable extension of bordering polymicrogyric cortex. The positron emission tomography scans of all patients revealed perisylvian metabolic abnormalities corresponding to the magnetic resonance imaging-defined abnormality. Variable extent of abnormal glucose metabolism was also observed in areas with normal magnetic resonance imaging features. All patients with unilateral magnetic resonance imaging abnormality exhibited abnormal glucose metabolism also in the contralateral side. The two patients with bilateral malformation had more extensive positron emission tomography abnormalities than the morphologic anomalies on MRI. Although MRI remains the diagnostic gold standard to detect the lesion, positron emission tomography scan is helpful to evaluate the full functional extension of the cortical anomaly, thereby contributing to the definition of the clinical severity of the syndrome.
