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Table 2 comparison of neuroimaging features between RVCL-S, TD, and glioma

From: High clinical heterogeneity in a Chinese pedigree of retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S)

  CT T1WI T2WI/FLAIR Diffusion restriction Gd +  MRS Other adjuncts
RVCL-S Hypodense Hypointense Hyperintense Central Irregular rim NAA ↓ Family history ( +)
 ± calcification   Mixed   Punctate Cho↑ Multisystemic symptoms ( +)
Nodular Lip peak PET/CT may show decreased glucose uptake
None   CSF profiling is normal
Decreased cerebral blood perfusion on PWI
TD Hypodense Hypointense Peripheral hypointense rim with central hyperintense signal Peripheral Open ring (more common) NAA ↓ Ring enhancement co-localizing with T2W rim
Mixed   Closed ring Cho↑ Multiple ovoid lesions perpendicular to lateral ventricles
Patchy   Optic neuritis is more common than retinopathy
Cotton-ball   OCB is more likely to be positive
Nodular   PET/CT may show increased glucose uptake at the rim of the lesion with central hypometabolism
None   Decreased cerebral blood perfusion on PWI
Glioma Hypodense Hypointense Hyperintense Heterogeneous central Closed ring (more common) NAA ↓ High cerebral blood perfusion on PWI
Hyperdense Isointense Isointense Incomplete peripheral Central homogeneous Cho↑ Hemorrhagic lesion may present
Mixed Mixed Mixed   Central heterogeneous Lip peak PET/CT usually show increased glucose uptake
 ± calcification