MELAS syndrome: imaging and proton MR spectroscopic findings.
OBJECTIVE
To evaluate imaging findings in MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, strokes) syndrome for the presence and location of infarctions and the presence of lactate.


METHODS
Eight patients were studied with MR (n = 8) and CT (n = 2). One patient underwent single-photon emission CT with technetium 99m hexamethyl-propyleneamine oxime and one patient had conventional catheter angiography. One fixed brain was studied with MR imaging. Five patients underwent single volume proton MR spectroscopy. Imaging studies were evaluated for atrophy, edema, and infarctions. Proton MR spectroscopy was visually analyzed for presence or absence of lactate.


RESULTS
One patient showed a cerebral infarction, and later a second distant infarction developed. One patient showed a transient area of cortical edema. Two patients had small nonspecific periventricular white matter abnormalities and one patient had diffuse white matter hyperintensities. Two patients had nonspecific MR abnormalities (probably age-related changes), and two had normal MR findings. None had basal ganglia involvement. Proton MR spectroscopy showed presence of lactate in one case with transient cortical edema; in two cases with nonspecific (probably age-related) brain findings; and in two patients with normal MR findings.


CONCLUSIONS
Patients with MELAS have a variety of MR findings. The fact that proton MR spectroscopy showed lactate in all five cases studied, regardless of MR findings, indicates that proton MR spectroscopy may be more sensitive in the detection of MELAS-associated abnormalities than MR imaging.
Castillo
M
M
Department of Radiology, University of North Carolina at Chapel Hill 27599-7510, USA.
Kwock
L
L
Green
C
C
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D001923:Brain Chemistry; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007773:Lactates / Q000032:analysis; D019344:Lactic Acid; D017241:MELAS Syndrome / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008875:Middle Aged; D015609:Organotechnetium Compounds; D010091:Oximes; D011859:Radiography; D019690:Technetium Tc 99m Exametazime; D015899:Tomography, Emission-Computed, Single-Photon
D007773:Lactates; D015609:Organotechnetium Compounds; D010091:Oximes; D019344:Lactic Acid; D019690:Technetium Tc 99m Exametazime