Bilateral vertebral artery dissection, agenesis of both ICAs, and connective tissue aberrations

A 35-year-old woman presented with acute signs of stroke (appendix e-1 on the Neurology® Web site at www.neurology.org). After initial CT with angiography, MRI with time-of-flight angiography confirmed agenesis of both internal carotid arteries (ICAs; figure 1A). It revealed bilateral vertebral artery (VA) dissections and ischemias in both middle artery territories (figure 1, A–E). Skin biopsy microscopy (figure 2) was consistent with ultrastructural connective tissue disease (uCTD), for which no further evidence was found apart from mild hypermobility of the finger joints. The underlying uCTD with structural instability of the arterial walls and the increased blood flow in the vertebrobasilar circulation due to the bilateral ICA agenesis may have promoted VA dissection.

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Figure 1 MRI with time-of-flight angiography shows bilateral internal carotid artery agenesis, vertebral artery dissections, and ischemias in both middle artery territories

Time-of-flight MRI angiography shows absence of both internal carotid arteries (A, upper arrows) and enlarged vertebral arteries (VAs) providing compensatory blood supply (lower arrows). It further reveals bilateral VA dissections, with cervical dissection in segment V2 on the right (B) and in segment V3 on the left (C). Ischemic lesions in both middle artery territories are visible (D, E).

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Figure 2 Electron microscopy of the skin biopsy shows evidence of ultrastructural connective tissue aberrations

Electron microscopy of the skin biopsy (A, magnification ×35,000) shows loosely packed collagen bundles with caliber variability of fibrils (arrows) consistent with an ultrastructural connective tissue syndrome. (B) Historical control for comparison purposes.

• © 2013 American Academy of Neurology