Which imaging findings may be helpful in differentiating Sturge-Weber syndrome (SWS) from other conditions?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Imaging findings in differential diagnosis

Neuroimaging findings similar to those of Sturge-Weber syndrome may be found in several conditions. For example, in Dyke-Davidoff-Masson syndrome, one cerebral hemisphere is partially or completely atrophic as a result of an intrauterine or perinatal carotid artery infarction. Since the cerebral atrophy in Sturge-Weber syndrome also occurs during infancy, changes similar to those of the Dyke-Davidoff-Masson syndrome, including cerebral hemiatrophy with ipsilateral calvarial diploic space enlargement, may be seen.

Severe siderosis, prior to the injection of contrast material, demonstrates MRI findings similar to those seen in Sturge-Weber syndrome with cerebral hemiatrophy. However, the typical contrast enhancement and the abnormal veins seen with contrast injection easily separate these 2 conditions.

Calcification secondary to intrathecal methotrexate therapy and meningitis must be included in the differential diagnosis of cortical pattern calcification, when this is viewed on CT scans. However, neither of these would demonstrate the unilateral specific geographic localization.

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