CNS Vasculitis in Autoimmune Disease: MR Imaging Findings and Correlation with Angiography
Martin G. Pompera,
Timothy J. Millera,
John H. Stonea,
William C. Tidmorea and
David B. Hellmann
,a
a From the Departments of Radiology (M.G.P.) and Medicine (J.H.S., W.C.T., D.B.H.), The Johns Hopkins University School of Medicine, The Johns Hopkins Hospital, Baltimore; and the Department of Radiology (T.J.M.), Good Samaritan Hospital, Cincinnati.
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FIG 1. Patient 1: 60-year-old woman with primary angiitis of the CNS (a typical case of CNS vasculitis).
A and B, Axial SE MR images (3000/34). In A, an infarct is seen in the right subcortical white matter and the deep white matter (posterior MCA distribution) (arrow). Lacunar infarcts are also present in the globus pallidi (arrowheads). In B, infarcts are present in the left subcortical white matter (PCA distribution) (white arrows) and posterior left hippocampus (black arrow). Increased signal in the putamina and midbrain are artifactual.
C, Right lateral digital subtraction angiogram reveals mild narrowing of the proximal (near the A2 segment bifurcation) and several distal pericallosal artery segments (arrows). The MCA is poorly visible.
D, Anteroposterior digital subtraction angiogram of the right common carotid artery shows significant narrowing of the right M2 segment (arrows), with consequent attenuation of the more distal segments. The left MCA was normal.
E and F, Anteroposterior left vertebral artery (E) and lateral left common carotid artery (F) injections depict a fetal origin of the left PCA, with beading and occlusion of the PCA (arrows in F) and correlate with the MR image shown in B.
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FIG 2. Patient 2: CNS vasculitis in a 37-year-old woman with systemic lupus erythematosus.
A, Axial SE MR image (3000/90) shows infarcts in the right cortical gray matter and subcortical white matter (MCA distribution, upper arrow; MCA/PCA watershed distribution, lower arrow).
B, Axial SE MR image (3000/90) shows infarcts in the left cortical gray matter and subcortical white matter (PCA distribution, arrow).
C, Angiogram of the left internal carotid artery shows areas of stenosis in the ACA, MCA, and PCA distributions (arrowheads).
D, Angiogram of the right internal carotid artery shows lesions in the ACA (pericallosal) (arrowheads) and severe narrowing of the distal MCA (arrow). Note abnormal bilateral ACAs without corresponding MR abnormality. The MR image was obtained 7 days before the angiogram.
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FIG 3. Patient 3: Hemorrhage in CNS vasculitis.
A, Axial SE MR image (550/25) shows T1 bright signal, consistent with hemorrhage in the left putamen (large straight arrow), left insular cortex (curved arrow), and in the region of the right external capsule (small arrows).
B, The corresponding T2-weighted SE image (3000/90) depicts bright signal in the basal ganglia bilaterally and particularly in the right caudate head (large arrow), external capsule (small arrows), and posterior limb, internal capsule (arrowhead). Abnormalities are also present in the thalami, right frontal cortex, and left periatrial white matter. The T2 bright regions are attributable to ischemia or infarction.
C, Angiogram of the right common carotid artery reveals attenuated flow in distal segments of the MCA caused by stenoses in the M1 segment (double arrow) and near the MCA trifurcation (single arrow).
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FIG 4. Patient 4: 71 year-old woman with primary angiitis of the CNS.
A and B, Axial FLAIR MR images (11,004/114/2200) show foci of increased signal intensity at the anterior portions of the external capsules bilaterally (A, arrows) and decreased signal adjacent to the anterior right ventricle (B, curved arrow).
C and D, Corresponding T2-weighted SE images (3000/91) show the lesion in the anterior right ventricle (D, curved arrow) and an additional lesion in the right thalamus (C, arrow).
E–G, Angiograms depict lesions in right M1 (E, arrows), left A2 and M2 (F, long arrow and short arrow, respectively), in both PCAs (G, short arrows), and in left anterior inferior cerebellar artery (G, long arrow).
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FIG 5. Patient 5: 41-year-old woman with primary angiitis of the CNS and SAH over the left cerebral convexity.
A, CT scan shows SAH (arrows).
B, SE MR image (3000/100) corresponding to the CT scan in A fails to reveal any parenchymal abnormality; however, decreased T2 signal is present in the region corresponding to subarachnoid blood on the CT scan (arrows).
C, Coronal SE MR image (600/20) shows high signal intensity material that may be caused by hemorrhage over the left parietal lobe (arrow).
D, Angiogram shows multiple foci of dilatation and stenosis, predominantly in the left ACA distribution (arrowheads).
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FIG 6. Patient 6: 24-year-old woman with primary angiitis of the CNS.
A, Axial SE MR image (1500/30) shows high signal intensity in the left parietal cortex, subcortical white matter (black arrows), and in the left putamen (white arrow).
B, Contrast-enhanced axial SE MR image (550/25) shows vascular and mild parenchymal enhancement (arrows). The vascular stasis is indicative of acute cerebral ischemia.
C, Right internal carotid artery angiogram shows narrowing in the right pericallosal artery (arrowheads). Superimposition of vessels immediately distal to that lesion gives the appearance of an aneurysm.
D, Left common carotid artery angiogram reveals attenuated MCA branches distal to a trifurcation aneurysm (arrowhead).
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