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Sturge-Weber syndrome: diffusion magnetic resonance imaging and proton magnetic resonance spectroscopy findings

S Cakirer1, B Yagmurlu, M R Savas

  • 1Department of Radiology, Neuroradiology Section, Istanbul Sisli Etfal Hospital, Istanbul, Turkey. scakirer@yahoo.com

Acta Radiologica (Stockholm, Sweden : 1987)
|September 2, 2005
PubMed

Insights

Diffusion MRI and MR spectroscopy reveal restricted diffusion and neural tissue disintegration in a patient with Sturge-Weber syndrome. These findings suggest neuronal loss and water molecule motion in affected brain regions.

Area of Science:

  • Neuroimaging
  • Neurology
  • Radiology

Background:

  • Sturge-Weber syndrome is a rare congenital disorder characterized by a facial birthmark and neurological abnormalities.
  • Diffusion magnetic resonance imaging (dMRI) and proton MR spectroscopy (MRS) are advanced neuroimaging techniques used to assess brain tissue integrity.

Observation:

  • A 26-year-old female patient with Sturge-Weber syndrome underwent diffusion MRI and proton MRS.
  • Echo-planar trace diffusion MRI showed increased signal intensity in the parieto-occipital lobes at b = 1000 s/mm2, indicative of restricted diffusion.

Findings:

  • Apparent diffusion coefficient (ADC) maps revealed moderately high signal intensity and elevated ADC values (0.9 x 10(-3) mm2/s) in affected areas compared to normal brain tissue (0.776 x 10(-3) mm2/s).
  • Proton MRS demonstrated reduced N-acetyl aspartate (NAA) and elevated choline (Cho) peaks, signifying neural tissue disintegration and neuronal loss.

Implications:

  • The observed dMRI and MRS findings suggest increased water molecule motion, consistent with disintegration of neural tissue in the parieto-occipital lobes.
  • These neuroimaging biomarkers can aid in understanding the pathophysiology and monitoring disease progression in Sturge-Weber syndrome.

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