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123 - Central Nervous System Vasculitis

from Section 4 - Abnormalities Without Significant Mass Effect

Published online by Cambridge University Press:  05 August 2013

Giulio Zuccoli
Affiliation:
University of Pittsburgh Medical Center
Zoran Rumboldt
Affiliation:
Medical University of South Carolina
Mauricio Castillo
Affiliation:
University of North Carolina, Chapel Hill
Benjamin Huang
Affiliation:
University of North Carolina, Chapel Hill
Andrea Rossi
Affiliation:
G. Gaslini Children's Research Hospital
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Summary

Specific Imaging Findings

Imaging features indicative of Central Nervous System Vasculitis (CNSV) are multiple infarcts of various ages in more than one vascular territory. CT images may be negative in the hyperacute phase and multifocal low-density lesions may be depicted in the acute–subacute phase. Localized subarachnoid hemorrhage is occasionally seen. On MRI, CNSV is characterized by scattered areas of high T2 and low T1 signal intensity in the deep gray matter, cortical and subcortical regions, which may be quite nonspecific. In the acute–subacute phase dark foci of cytotoxic edema are present on ADC maps. Increase in ADC values in the otherwise normal-appearing white matter may represent vasogenic edema, chronic destruction, and/or Wallerian degeneration. Microhemorrhages are frequently present on T2*-weighted images, subarachnoid blood is sometimes seen, usually along the cortical sulci. Scattered areas of leptomeningeal enhancement with underlying brain signal abnormalities are highly characteristic for vasculitis, but are seen in only about a third of cases. Wall thickening and enhancement of large intracranial vessels is a diagnostic sign of CNSV, present in most patients. Classic angiographic findings are diffuse segmental arterial wall narrowing and associated poststenotic dilation, referred to as “beading” or “string-of-beads”. Multi-vessel involvement increases the suspicion for CNSV. When more than two stenoses in at least two separate vascular distributions are depicted on MRA, DSA is not needed.

Pertinent Clinical Information

CNSV can affect any age group, but is more common in individuals 40–60 years old. Clinical manifestations are nonspecific, including headache, encephalopathy and, less frequently, focal neurological deficits. Diagnosis is often very difficult. Cerebral catheter angiography has been the standard for presumed vasculitis, with sensitivity in the range of about 80%, while MRA (and CTA) are slightly less sensitive. Cerebral ormeningeal biopsy is the gold standard but also has limited sensitivity. A combination of steroids, immunosuppressants, and immunomodulating agents is used for treatment.

Type
Chapter
Information
Brain Imaging with MRI and CT
An Image Pattern Approach
, pp. 253 - 254
Publisher: Cambridge University Press
Print publication year: 2012

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References

1. Zuccoli, G, Pipitone, N, Haldipur, A, et al. Imaging findings in primary central nervous system vasculitis. Clin Exp Rheumatol 2011;29:S104–9.Google ScholarPubMed
2. Kuker, W, Gaertner, S, Nagele, T, et al. Vessel wall contrast enhancement: a diagnostic sign of cerebral vasculitis. Cerebrovasc Dis 2008;26:23–9.Google ScholarPubMed
3. White, ML, Hadley, WL, Zhang, Y, Dogar, MA. Analysis of central nervous system vasculitis with diffusion-weighted imaging and apparent diffusion coefficient mapping of the normal-appearing brain. AJNR 2007;28:933–7.Google ScholarPubMed
4. Demaerel, P, De Ruyter, N, Maes, F, et al. Magnetic resonance angiography in suspected cerebral vasculitis. Eur Radiol 2004;14:1005–12.CrossRefGoogle ScholarPubMed
5. Hajj-Ali, RA, Singhal, AB, Benseler, S, et al. Primary angiitis of the CNS. Lancet Neurol 2011;10:561–72.CrossRefGoogle ScholarPubMed

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