PMC4857362_01
(a and b) Axial DWI sequence MRI demonstrating scattered punctate ischaemic lesions in the left temporal lobe, arrows. (c and d) Axial FLAIR MRI showing multiple areas of cortical hyperintensity in the left hemisphere, block arrows. (e) Intracranial MRA demonstrating normal intracranial vessels.. DWI: diffusion weighted imaging; MRI: magnetic resonance imaging; FLAIR: fluid-attenuated inversion recovery; MRA: magnetic resonance angiography.
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Unusually, for ischaemic stroke, there were also prominent areas of cortical fluid-attenuated inversion recovery (FLAIR) hyperintensity and thickening of the cortex (Figure 1).
(a and b) Axial DWI MRI performed 9 days after Figure 1, revealing multiple new infarcts with left hemisphere predominance, arrows. (c and d) Axial gradient echo sequence showing multiple foci of increased susceptibility suggesting micro-haemorrhage, in addition to a linear area of subarachnoid haemorrhage in the left frontal region, block arrows. (e) Axial T1-weighted MRI with gadolinium showing leptomeningeal enhancement predominantly in the left temporal region, arrowheads.. DWI: diffusion weighted imaging; MRI: magnetic resonance imaging.
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Repeat imaging 1 week later showed further progression with multiple new ischaemic lesions, extensive leptomeningeal enhancement restricted to the left hemisphere, particularly the left temporal lobe, as well as subcortical micro-haemorrhages and minor convexity subarachnoid blood which were not present on the first MRI (Figure 2).
H&E stains from the left temporal lobe brain biopsy showing typical changes of PACNS. (a and b) Fibrinoid change in a superficial cortical small muscular blood vessel, arrow, as well as perivascular inflammatory infiltrate, predominantly lymphocytic with occasional histiocytes and multinucleated giant cells, block arrow, 40x and 200x magnification. (c and d) Area of cortical infarction, 40x and 100x magnification.. H&E: haematoxylin and eosin; PACNS: primary angiitis of the central nervous system.
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The findings were consistent with PACNS - showing patchy infarcts of variable age and vasculitic changes in small muscular cerebral and meningeal blood vessels consisting of transmural infiltrates predominantly of lymphocytes with fibrinoid change or infarction of the vessel walls (Figure 3).