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Research paper
Subtypes of primary angiitis of the CNS identified by MRI patterns reflect the size of affected vessels
  1. Simon Schuster1,
  2. Henrike Bachmann1,
  3. Vivien Thom1,
  4. Ann-Katrin Kaufmann-Buehler2,
  5. Jakob Matschke3,
  6. Susanne Siemonsen2,
  7. Markus Glatzel3,
  8. Jens Fiehler2,
  9. Christian Gerloff1,
  10. Tim Magnus1,
  11. Götz Thomalla1
  1. 1 Department of Neurology, University Hospital Hamburg-Eppendorf, Germany
  2. 2 Department of Diagnostic and Interventional Neuroradiology, University Hospital Hamburg-Eppendorf, Germany
  3. 3 Institute of Neuropathology, University Hospital Hamburg-Eppendorf, Germany
  1. Correspondence to Simon Schuster, Department of Neurology, University Hospital Hamburg-Eppendorf, Martinistraße 52, 20246 Hamburg, Germany; s.schuster{at}


Objective To describe patterns of diagnostic findings, and identify subgroups of primary angiitis of the central nervous system (PACNS).

Methods We retrospectively analysed 31 patients with PACNS. Cases were selected by predetermined diagnostic criteria and stratified into biopsy-proven and imaging-based PACNS. We compared clinical characteristics, cerebrospinal fluid (CSF) findings and imaging results including high-resolution vessel wall MRI between groups.

Results There were 31 cases of PACNS (mean age 45.6 years, 58.1% female), of whom 17 (55%) were biopsy-proven, 14 (45%) were based on imaging findings. Patients with a positive biopsy had fewer infarcts (29.4% vs 85.7%, p=0.003), were more likely to have meningeal and parenchymal contrast enhancement (76.5% vs 28.6%, p=0.012), were less likely to have abnormal MR angiography (11.8% vs 100%, p<0.001) and did not show vessel wall enhancement at the time of diagnosis (0% vs 76.9%, p<0.001). In contrast, patients with imaging-based diagnosis showed more frequently multiple infarcts and vessel abnormalities, with vessel wall enhancement in most of the cases. Clinical characteristics and CSF analysis did not reveal marked differences between groups.

Interpretation Multi-parametric MRI distinguishes two subtypes of PACNS that most likely differ concerning the affected vessel size. Biopsy-proven PACNS primarily involves smaller vessels beyond the resolution of vascular imaging, while imaging-based PACNS affects predominantly medium-sized vessels leading to false-negative biopsy results. Using distinct MRI patterns may be helpful for selecting patients for appropriate invasive diagnostic modalities.

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  • Contributors SS and HB: study design, data acquisition, analysis and interpretation of data, and drafting the manuscript. TV and JF: revising the manuscript. A-K K-B and SS: data acquisition and revising the manuscript. JM: data acquisition, analysis and interpretation of data, and revising the manuscript. MG: analysis and interpretation of data, and revising the manuscript. CG, TM and GT: study concept and design, analysis and interpretation of data, and revising the manuscript.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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