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Cerebral vasculitis: imaging signs revisited

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Abstract

Inflammatory stenoses of cerebral blood vessels, although rare in general, are an important cause of cerebral ischemia in younger patients. The diagnosis is often difficult. The first step in the diagnostic process is the identification of brain lesions consistent with cerebral vasculitis. Brain lesions are frequently found in this patient group, especially if modern imaging tools such as diffusion and perfusion-weighted imaging are employed. Although no specific pattern for this entity exists, multiple infarcts of various ages in more than one vascular territory should raise this suspicion. The next step in the imaging of patients with suspected vasculitis is the demonstration of the underlying vascular pathology. MR angiography is the mainstay of investigating patients for intracranial vascular stenoses. However, at 1.5 T it is only diagnostic for stenoses of large brain arteries. Hence, conventional angiography is still required to investigate stenoses of medium and small-sized brain arteries. Recent work suggests that MRI can directly demonstrate mural thickening and contrast enhancement in basal brain arteries, rendering biopsy obsolete in this patient group. A classification for cerebral vasculitis is proposed according to the size of the affected brain vessels, analogous to the pertinent nomenclature of primary systemic vasculitis.

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I declare that I have no conflict of interest.

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Authors and Affiliations

  1. Department of Neuroradiology, West Wing, The John Radcliffe Hospital, Headley Way, Oxford, OX3 9DU, UK

    Wilhelm Küker

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  1. Wilhelm Küker
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Correspondence to Wilhelm Küker.

Suggested diagnostic protocol for cerebral vasculitis

Suggested diagnostic protocol for cerebral vasculitis

Initial imaging: MRI

Mandatory

T2, T2* and DWI images of the whole brain. TOF-MRA of the large brain arteries and post-contrast T1-weighted images of the brain parenchyma. High-resolution post-contrast T1-weighted images (3 mm or less) of areas of abnormality on MRA. Vessel wall enhancement should be demonstrated in two planes. Axial sequences should be performed using fat suppression and flow compensation.

Optional

Perfusion-weighted imaging, CE-MRA.

Secondary imaging: cerebral angiography

Mandatory

Selective injections of both the internal carotid artery and at least one vertebral artery. Small field of view (25 cm or less), high spatial and temporal resolution (at least two frames per second) imaging of medium sized brain arteries in at least two planes.

Optional

A biplane angiography unit significantly reduces the amount of contrast material needed.

If imaging is inconclusive: brain or leptomeningeal biopsy.

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Küker, W. Cerebral vasculitis: imaging signs revisited. Neuroradiology 49, 471–479 (2007). https://doi.org/10.1007/s00234-007-0223-3

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