What is the role of SPECT scanning in the workup of Sturge-Weber syndrome (SWS)?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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This modality measures cerebral blood flow and demonstrates underperfusion in the area of the pial angioma; it may therefore detect a latent angioma not seen in other studies (see the image below). Using SPECT scanning, Reid et al demonstrated the presence of hypoperfusion before calcifications, anomalous drainage, or enhancement developed on either CT or MRI scans. [23] Griffiths et al showed that MRI and SPECT scanning together may reveal different areas of involvement. [78]

Single-photon emission computed tomographic scan i Single-photon emission computed tomographic scan in Sturge-Weber syndrome.

Namer et al demonstrated a steal phenomenon during seizures, causing ischemia in remote areas, with subtraction ictal SPECT co-registered to MRI (SISCOM). [79]

Pinton et al demonstrated that in infants with SWS, the cortex is hyperperfused during the first year of life before the first seizures occur. Classic hypoperfusion appears after 1 year of age, even in patients without epilepsy. [80]

Maria et al reported that enlargement of the choroid plexus correlates with abnormalities seen with SPECT scanning. [1]

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