PMC5966840_01
Complementary examinations: Brain CT angiography (a) and brain arteriography (b): On brain CT angiography, there was an ectatic aspect of the distal branch of the right middle cerebral artery (arrow on (a)). Cerebral catheter angiography found a focal fusiform enlargement of the distal cervical portion of the right internal carotid artery, related to a pseudo-aneurysm suggesting an evolution of a dissection, above which a string of beads suggesting a dysplasic artery (arrow on (b)). Brain MRI: (c) T2-weighted FLAIR sequence, axonal slice: bilateral frontal lobe hypersignal. Moderate mass effect. (d) T2-weighted gradient echo sequence: numerous punctiform cortical hyposignals adjacent to white matter infiltration regions. (e) Spectrometry, TE (echo time) 35 ms: normal NAA/Cr and Cho/Cr ratios, slight lipid-lactate peak at 1.33 ppm. (f) Color map, perfusion-weighted sequence: reduced rCBV in FLAIR hypersignal region. (g) Control MRI after 3 months of corticotherapy: T2-weighted FLAIR sequence, axonal slice, showing clear regression of white matter hypersignal, compared to initial images. Right frontal cerebral biopsy (arrow on (c)): (h) arachnoid and cortical vessels stained by Congo red. Polarized light: green birefringence of amyloid deposits.
10.1177_2050313X18777176-fig1.jpg
PMC5966840_01_10.1177_2050313X18777176-fig1.jpg
fig1-2050313X18777176
CT angiography, there was an ectatic aspect of the distal branch of the right middle cerebral artery (Figure 1(a)). Cerebral catheter angiography found a focal fusiform enlargement of the distal cervical portion of the right internal carotid artery, related to a pseudo-aneurysm suggesting an evolution of a dissection, above which a string of beads suggesting a dysplastic artery (Figure 1(b)). T2-weighted gradient echo sequences. There was no contrast uptake and moderate mass effect (Figure 1(c) and (d)). There was however a slight lipid-lactate peak at 1.33 ppm (Figure 1(e)). On perfusion sequences, relative cerebral blood volume (rCBV) was low (Figure 1(f)). Right frontal cerebral biopsy (arrow on Figure 1(c)) found thick-walled arachnoid and cortical vessels, without perivascular inflammatory infiltrate, granuloma or giant cells. Specific staining revealed congophilic amyloid deposits (Figure 1(h)). CSF analysis was normal, tau protein levels had decreased to 241 ng/L, and beta-amyloid 1-40 and 1-42 levels were stable, respectively, 10.37 and 443 ng/L. Control brain MRI showed clear regression of FLAIR hypersignals (Figure 1(g)).