Which clinical procedures are used to confirm the diagnosis of Sturge-Weber syndrome (SWS)?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
  • Print
Answer

Besides the clinical examination, the following have historically been the procedures of choice to establish the diagnosis (see Table 3, below) [1] :

  • Angiography

  • CT scanning

  • MRI

  • MRI with gadolinium

  • Functional imaging - With SPECT or PET scanning

Table 3. Summary of Work-up Findings in Sturge-Weber Syndrome (Open Table in a new window)

Procedure

Findings

CSF analysis

Elevated protein

Skull radiography

Tram-track calcifications

Angiography

Lack of superficial cortical veins

Non-filling dural sinuses

Abnormal, tortuous vessels

CT scanning

Calcifications, tram-track calcifications

Cortical atrophy

Abnormal draining veins

Enlarged choroid plexus

Blood-brain barrier breakdown (during seizures)

Contrast enhancement

MRI

Gadolinium enhancement of leptomeningeal angiomas (LAs)

Enlarged choroid plexus

Sinovenous occlusion

Cortical atrophy

Accelerated myelination

SPECT scanning

Hyperperfusion, early

Hypoperfusion, late

PET scanning

Hypometabolism

Electroencephalography (EEG)

Reduced background activity

Polymorphic delta activity

Epileptiform features


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!