Skip to main content
. Author manuscript; available in PMC: 2022 Aug 1.
Published in final edited form as: Pediatr Neurol. 2021 May 6;121:59–66. doi: 10.1016/j.pediatrneurol.2021.04.013

Table 2.

Key Points for Neuroradiological Management and Treatment in SWS

1A. In newborns and infants with a high-risk PWB and no history of seizures or neurological symptoms, routine screening for brain involvement is not recommended.
1B. In newborns and infants with a high-risk PWB and no history of seizures or neurological symptoms, brain imaging can be performed in select cases, e.g., in those where presymptomatic treatment is being considered. Indirect signs of SWS brain involvement and susceptibility-weighted imaging can optimize the yield of MRI in this group.
2A. In children with suspected SWS, the first post-symptomatic imaging should be an optimized pre- and post-contrast MRI sequence, focusing on the detection of both vascular and parenchymal abnormalities associated with SWS.
2B. Routine follow-up neuroimaging is not recommended in children with established SWS and stable neurocognitive symptoms. In case of progressive neuro-cognitive symptoms during follow-up, multisequence brain MRI comparable to previous imaging should be performed for an accurate comparison.
2C. Neuroimaging should be obtained in adults with PWB with or without glaucoma who have not had prior imaging; however, follow-up neuroimaging is not recommended in adults with established SWS and stable neurocognitive symptoms. Pre- and post-contrast brain MRI is recommended in case of new-onset or progressive symptoms.
3A. SWS patients rarely hemorrhage and there is little evidence for acute ischemia. The available data do not show bleeding or clear strokes in SWS patients who present with acute neurological symptoms; however, prolonged new-onset or acutely/subacutely deteriorating and non-resolving neurological ‘stroke-like’ episodes justify repeat neuroimaging.
4A. Presurgical evaluation in SWS can benefit from the use of advanced structural and functional imaging modalities. These studies and the subsequent surgery should be performed in specialized pediatric epilepsy centers that are experienced in processing and interpretation of advanced imaging and also in surgical techniques used in SWS (such as hemispherectomy).