Imaging of Glutamate Concentration in Sturge-Weber Syndrome

معرفی دختر مبتلا به سندرم استورج وبر همراه با بدشکلی استخوانی در ناحیه دهان- فک بالا- صورت

Background: Sturge-Weber syndrome is one of the rare neurocutaneous disorders with frequency of approximately 1 per 50,000. Sturge-Weber syndrome consists of a constellation of symptoms and signs including a facial nevus (port wine stain), seizure and hemiparesis. In many cases it may associate with mental retardation. Case presentation: A 7- year old girl with mental retardation and a large fa...

Genetic interaction between Fmr1 and Grm5: A role for mGluR5 in the pathogenesis of Fragile X Syndrome

Early characteristics of Sturge-Weber syndrome shown by perfusion MR imaging and proton MR spectroscopic imaging.

We report the case of a 9-month-old boy with Sturge-Weber syndrome and new onset of seizure. Perfusion MR imaging showed early changes compatible with impaired venous drainage in the affected hemisphere, whereas proton MR spectroscopic imaging revealed a focal parietal area of elevated choline without significant alteration of N-acetylaspartate levels. The perfusion and subtle metabolic abnorma...

Sturge-Weber syndrome: cranial MR imaging with Gd-DTPA.

PURPOSE To study the role of Gd-DTPA in cranial MR of patients with Sturge-Weber syndrome. METHODS AND MATERIALS Seven patients with Sturge-Weber syndrome, ages 3 months to 36 years, were evaluated with unenhanced and contrast-enhanced (Gd-DTPA) MR imaging. Correlation was made to cranial CT in five patients. RESULTS MR findings included (in decreasing frequency): pial angiomatosis, cerebra...

MR imaging of Sturge-Weber syndrome: role of gadopentetate dimeglumine and gradient-echo techniques.

Five patients with Sturge-Weber syndrome were evaluated by conventional noncontrast spin-echo MR imaging, a gradient-recalled echo (GRE) technique, and T1-weighted spin-echo imaging after administration of gadopentetate dimeglumine. In four of five cases the full extent of intracranial disease was appreciated only on the postcontrast images. In one patient precontrast and GRE images were entire...