Quantitative Assessment of Brain Networks in Children With Sturge-Weber Syndrome Using Resting State Functional Magnetic Resonance Imaging (MRI)

Abstract

In this study, we examined whether topologic network analysis, using resting state functional Magnetic Resonance Imaging (MRI), can detect abnormalities of functional brain connectivity in children with unilateral brain injury due to Sturge-Weber syndrome. Three children with Sturge-Weber syndrome (age: 1, 3, 10 years) underwent structural and resting state functional MRI, glucose metabolism Positron Emission Tomography (PET), and neurocognitive evaluation. Eight different resting state networks were compared between the affected and unaffected hemispheres by quantitatively accessing communication efficiency measures. Significantly reduced efficiency values were found in all three patients. Visual network deficiency was present in both children with a visual field defect; frontal network abnormalities were associated with fine motor impairment. Location of network abnormalities corresponded to and, in some cases, extended beyond structural MRI and glucose PET abnormalities. The presented approach can detect early functional abnormalities of specific brain networks in children with Sturge-Weber syndrome.

A Multidisciplinary Consensus for Clinical Care and Research Needs for Sturge-Weber Syndrome

“…Most advanced MRI techniques require further validation in prospective patient cohorts and should be acquired with as similar parameters as possible for multi-center studies. Examples include anatomic morphometry (including cortical thickness, gray and white matter volumes, 71 extent of calcifications 65 ), diffusion-weighted ADC and FA values, 60 structural connectivity from DTI, functional connectivity from resting-state fMRI, 72 perfusion parameters from DCE/DSC/ASL MRI, and various brain metabolites on MRS 73,74 (Table 2).…”

New vascular classification of port‐wine stains: improving prediction of Sturge–Weber risk

“… 5 , 9 – 11 Recent advances in functional imaging have demonstrated decreased cerebral perfusion and network connectivity in affected areas. 12 – 14 Clinical neurological problems include seizures (often but not always contralateral focal motor seizures), neurodevelopmental delay, headache and stroke-like episodes. 15 The ophthalmological features in SWS are enlarged venous vessels affecting the conjunctiva, episclera, retina and/or choroids, associated with glaucoma, retinal detachment and choroidal haemorrhage.…”

Intellectual and adaptive functioning in Sturge-Weber Syndrome

“…Lowered intelligence has also been associated with focal ipsilateral white matter tissue damage (Alkonyi et al, 2011), decreased white matter volume (Juhasz et al, 2007), unilateral brain damage (Behen et al, 2011), and the greater severity of the cerebral lesion (Pascual-Astroviejo et al, 2008) in SWS. Further, neural network abnormalities in SWS have been associated with their corresponding verbal cognition, motor, and visual field functions (Jeong, Chugani, Behen, Guy, & Juhasz, 2013). …”