Presentation
Worsening vertigo, ataxia, right leg weakness
Patient Data
Stable nonspecific patchy periventricular white matter hypoattenuation. No evidence of subacute or chronic infarction. CTA demonstrates multifocal stenoses of the right vertebral artery with narrower caliber throughout its length. There is little if any opacification of the proximal V4 segment. There is progressive reduced opacification of the V3 segment as it passes through the right C1 transverse foramen and around the posterior aspect of the right C1 lateral mass. There are multiple short segments of stenosis noted throughout the V1 and V2 portions. The left vertebral artery shows short segments of stenosis in the V1 and V2 segments and severe stenosis at the V3-V4 junction adjacent to the atlanto-occipital joint. The basilar artery is patent. The right and left PCAs are patent but of smaller caliber. Stenoses at the bilateral lacerum and cavernous ICA segments noted. Small probable left ophthalmic ICA aneurysm. The bilateral ACAs and MCAs are patent and of relatively preserved caliber.
No acute infarct. Bilateral tiny chronic cerebellar infarcts. Patchy foci of T2/FLAIR hyperintensity within the supratentorial white matter.
Case Discussion
The patient went on to have bilateral temporal artery biopsies showing: "Multiple small foci of chronic inflammatory cell infiltration around vasa vasora in the adventitia. Some inflammatory cells also appear to target the external elastic lamina. In one section, there is a narrow transmural scar with inflammatory cells, fibroblasts, myxoid collagen, focal fibrinoid material and hemorrhage; giant cells are not seen. The internal elastic lamina in most sections shows short discontinuous foci of fragmentation and focal reduplication with a mild intimal thickening. Although the pattern of inflammation is not entirely classical or fully developed, in this patient demographic features would be strongly suspicious for active temporal arteritis." Unfortunately, despite high dose immune suppression, the patient continued to deteriorate and two months later, with evidence of progressive arterial stenosis on imaging, passed away.
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