Pulsed arterial spin-labeled MR imaging evaluation of tuberous sclerosis

Background and Purpose: Tuberous sclerosis presents with characteristic cortical hamartomas and subependymal nodules associated with seizures. The purpose of this study was to use pulsed arterial spin-labeling (PASL) to quantify the perfusion of the cortical hamartomas and correlate the perfusion values with seizure frequency. MATERIALS AND METHODS: A retrospective search yielded 16 MR imaging examinations including conventional MR imaging and PASL perfusion performed in 13 patients (age range, 7 months to 23 years) with a history of tuberous sclerosis. The mean perfusion of each cortical hamartoma greater than 5 mm in size localized with conventional MR imaging sequences was obtained with use of manually drawn regions of interest. Cortical hamartomas were classified as normal, hyperperfused, or hypoperfused on the basis of the mean and SD of the unaffected cortex. Correlation was made between perfusion imaging, conventional imaging, and clinical history. Results: Of the 245 cortical hamartomas, 227 (92.7%) were hypoperfused, 10 (4.1%) were hyperperfused, and 8 (3.3%) were unchanged relative to the mean gray matter. One patient had a sub-ependymal giant cell astrocytoma with a mean perfusion of 93.5 mL/100 g tissue/min. There was a statistically significant positive correlation between seizure frequency and the number of hyperperfused cortical tubers (r = 0.51; n = 16; P = .04), with higher seizure frequency associated with a greater number of hyperperfused cortical tubers. There was no significant correlation, however, between seizure frequency and the overall number of cortical tubers (r = 0.20; n = 16; P = .47). Conclusions: The PASL technique can assess and quantify the perfusion characteristics of a cortical hamartoma. Most lesions are hypoperfused; however, both normally perfused and hyperperfused lesions occur. The presence of hyperperfused cortical tubers was associated with increased seizure frequency.