Source:Pediatric Neurology
Author(s): Matthew T. Whitehead, Michael Wien, Bonmyong Lee, Nancy Bass, Andrea Gropman
BackgroundMitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome is a mitochondrial disorder often causing progressive brain injury that is not confined to large arterial territories. Severe insults ultimately lead to gyral necrosis affecting the cortex and juxtacortical white matter; the neuroimaging correlate is partial gyral signal suppression on T2/FLAIR, resembling black toenails. We aim to characterize the imaging features and natural history of MELAS related gyral necrosis.Materials and MethodsDatabases at two children's hospitals were searched for brain MRIs from MELAS patients. Exams with motion artifact and those lacking T2/FLAIR sequences were excluded. Location, cumulative number, and maximum transverse diameter of necrotic gyral lesions were assessed using T2WI and T2/FLAIR sequences. Wilcoxon signed-rank test was employed to evaluate the relationship between disease duration and number of necrotic lesions.ResultsOne hundred twenty-four exams from 14 unique MELAS patients (16 +/- 3 years) were evaluated. Six of the 8 patients that developed brain lesions also developed gyral necroses (mean 13, range 0 to 44). Necrotic lesions varied in maximal diameter from 4 to 25 mm. Cumulative necrotic lesions correlated with disease duration (p<0.001).ConclusionThe black toenail sign signifying gyral necrosis is a common imaging feature in MELAS syndrome. Gyral necrosis extent correlates with disease duration.
http://ift.tt/2t6nlHE
Δεν υπάρχουν σχόλια:
Δημοσίευση σχολίου