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The spectrum of CNS vasculitis in children and adults

Abstract

Children and adults who present with severe, newly acquired neurological or psychiatric deficits should be evaluated for an underlying inflammatory brain disease—the inflammation could be reversible, if diagnosed and treated rapidly. In our experience, primary angiitis of the central nervous system (PACNS) is the most common inflammatory brain disease and is increasingly recognized across patients of all ages. Distinct disease subtypes have been reported with characteristic disease courses, neuroimaging features and histopathological findings. In this Review, we provide a comprehensive comparison of childhood and adult PACNS, revealing distinct gender distributions, characteristic presenting clinical phenotypes and tailored differential diagnosis evaluations in the different subtypes of PACNS. Novel and traditional laboratory markers can help to define disease subtype and activity, whilst MRI and angiography can aid diagnosis in both children and adults. Characteristic patterns of parenchymal lesions and vessel involvement have been identified in PACNS and differ markedly between subtypes. Brain histopathology has also revealed distinct inflammatory pathways at different ages. Immunosuppressive treatment protocols have been shown to be effective and safe across the age spectrum; overall, in the past few years, the mortality of PACNS has decreased dramatically.

Key Points

  • Inflammatory brain diseases can affect males and females of all ages; in our experience, primary angiitis of the central nervous system (PACNS) is the most common type

  • Distinct disease subtypes have been identified in children and adults with PACNS and found to be associated with typical demographic characteristics

  • Newly acquired neurological or psychiatric deficits in children and adults mandate an evaluation for an underlying inflammatory brain disease

  • Diagnosis of inflammatory brain diseases should include a thorough clinical evaluation, targeted laboratory investigations and specific parenchymal and vascular neuroimaging techniques, including vessel wall imaging and, if required, an elective brain biopsy

  • The comparison of PACNS in children and adults has revealed major differences in classification strategies, clinical phenotypes, inflammatory markers, neuroimaging characteristics and inflammatory pathways

  • Evaluated treatment regimens are available for childhood PACNS and treatment recommendations are now available for adult PACNS

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Figure 1: Neuroimaging of nonprogressive primary CNS vasculitis and stroke.
Figure 2: Neuroimaging of progressive primary CNS vasculitis.
Figure 3: Neuroimaging and brain histopathology of small-vessel primary CNS vasculitis.
Figure 4: Diagnostic algorithm for suspected inflammatory brain diseases in children.

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Acknowledgements

The histopathology images were kindly provided and reviewed by Dr C. Hawkins from The Hospital for Sick Children, Toronto, Canada.

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M. Twilt researched data for the article. Both authors contributed equally to discussion of the content, writing, reviewing and editing the manuscript.

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Correspondence to Susanne M. Benseler.

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Twilt, M., Benseler, S. The spectrum of CNS vasculitis in children and adults. Nat Rev Rheumatol 8, 97–107 (2012). https://doi.org/10.1038/nrrheum.2011.197

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