Case study on Sturge-Weber syndrome

Dr. Christina Andica and Dr. Akifumi Hagiwara, Juntendo University School of Medicine, Tokyo, Japan

Sturge-Weber syndrome (SWS) is a developmental disorder with leptomeningeal angiomatosis as the major pathological abnormality. Typically, a prominent hypo-intensity on T2W images is observed in the white matter underlying the angiomatosis in an infant with SWS. In this example a 4 month-old male infant was referred to our hospital with a few episodes of left leg twitching. Clinical examination showed a right facial angiomatosis and a left leg hemiparesis. SyMRI was acquired on a 3T Discovery 750 system (GE Healthcare).

To be able to view a high image contrast for pediatric brains with higher water content, more extreme settings for the synthetic images were chosen than typical for conventional images. For example, for T2W we used TE/TR = 150/15000 ms, for  T1W a TE/TR = 10/100 ms and for the Double IR a TE/TR = 10/6000 ms. Since all images are derived from the same sequence there is no scan time penalty to do so (see Figure 1).

The synthetic DIR clearly demonstrated myelinated white matter. Accelerated myelination was confirmed by the myelin map, where a substantial left-right difference was observed (Figure 1D). The myelin volume in the right hemisphere was 7.9 ml, in comparison to the myelin volume in the left hemisphere of 4.2 ml. The use of SyMRI facilitates the diagnosis of SWS because it both provides high-contrast images and objective measurements. Quantitative detection and follow-up may improve the prognosis by preventive anti-epileptic treatment.

 

Figure 1. Representative slice for a 4 month-old patient with Sturge-Weber syndrome. In order to enhance image contrast, extreme settings for the synthetic images were chosen. A) T2W (TE/TR = 150/15000 ms), B) T1W (TE/TR = 10/100 ms) and C) Double IR (TE/TR = 10/6000 ms with TI1 = 860 ms and TI2 = 3490 ms).

The myelin map (D) shows the hypermyelination in the right hemisphere.

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