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Updated: Aug 23, 2026

Functional Magnetic Resonance Imaging (fMRI) of the Visual Cortex with Wide-View Retinotopic Stimulation
Published on: December 8, 2023
Visual functional magnetic resonance imaging in patients with Sturge-Weber syndrome
1Department of Radiology, Miami Children's Hospital, Miami, FL 33155, USA.
Insights
Sturge-Weber syndrome patients show varied visual cortex activation patterns. Functional MRI reveals that despite vascular malformations, cortical activation is possible, aiding surgical and counseling decisions.
Area of Science:
- Neuroscience
- Pediatric Neurology
- Radiology
Background:
- Sturge-Weber syndrome is a congenital disorder characterized by a facial birthmark and abnormalities in the brain and blood vessels.
- Understanding visual cortex function in these patients is crucial for clinical management.
Observation:
- Functional magnetic resonance imaging (fMRI) was used to study visual cortex activation in three children with Sturge-Weber syndrome.
- Activation patterns were compared to those of eight sedated children and six awake adult volunteers using a flashing lights paradigm.
Findings:
- Adult volunteers showed bilateral activation in primary and secondary visual cortex (Brodmann's areas 17, 18, and 19).
- Sedated children primarily exhibited primary visual cortex activation, with one showing no activation.
- Sturge-Weber syndrome patients displayed diverse patterns: increased activation, no activation, or abnormal distribution in the affected occipital lobe.
Implications:
- The vascular malformations in Sturge-Weber syndrome do not necessarily preclude cortical activation.
- Abnormal activation patterns in the visual cortex are associated with Sturge-Weber syndrome.
- fMRI assessment of cortical function can inform surgical interventions and patient counseling for Sturge-Weber syndrome.
Abstract:
The purpose of this study is to report different patterns of visual cortex activation in patients with Sturge-Weber syndrome as compared with healthy control subjects. Utilizing a visual paradigm of flashing lights, three children with Sturge-Weber syndrome were studied with functional magnetic resonance imaging. The results are compared with those documented in eight normal sedated children, and six young adult awake volunteers, using the same paradigms. All adult volunteers manifested bilateral activation in primary visual cortex (Brodmann's 17 and 18 areas). Two of them also had activation in secondary visual cortex (Brodmann's 19 area). In the eight sedated normal children, seven manifested activation in primary visual areas. The last exhibited no activation. The patients with Sturge-Weber syndrome demonstrated in the affected occipital lobe increased activation in one patient (11 months old), no activation in the second (12 years of age), and abnormal distribution of the activation in the third (11 months old). This report demonstrates that the vascular malformation of Sturge-Weber syndrome does not necessarily prevent cortical activation in the expected occipital cortex and may be associated with different patterns of abnormal activation. Assessing cortical function with functional magnetic resonance imaging in patients with Sturge-Weber syndrome may be helpful in decisions of surgical management and counseling.

