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Review
. 2024 Apr;116(2):134-139.
doi: 10.32074/1591-951X-987.

Primary angiitis of the central nervous system

Francesca Gianno  1 , Manila Antonelli  1 , Antonio d'Amati  2   3 , Giuseppe Broggi  4 , Angela Guerriero  5 , Alessandra Erbetta  6 , Luigi Caputi  7 , Gianluca Marucci  8
Affiliations
Review

Primary angiitis of the central nervous system

Francesca Gianno et al. Pathologica. 2024 Apr.

Abstract

Primary angiitis of the central nervous system (CNS) is an uncommon inflammatory disorder, with highly variable clinical presentation. It needs to be differentiated from several mimickers, such as CNS involvement in systemic vasculitides, connective tissue disorders, infectious disease, and leukodystrophy as well as neoplastic diseases. The diagnosis requires a combination of clinical and laboratory investigations, multimodal imaging, and histopathological examination, which should be available for confirmation. In the present paper, the histopathological features of primary angiitis of the CNS are described and highlighted to help pathologists avoid misdiagnosis of a treatable acquired disease.

Keywords: brain biopsy; cerebral ischemia; cerebral vasculitis; primary angiitis of the central nervous system.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1.
Figure 1.
Axial T2 w.i. (A, B) and T2* w.i. (C, D). Note in A and B multiple asymmetric swollen white matter lesions; no areas of bleeding are seen. Microhemorrhages (arrows) are detected on T2*. Histological examination evidenced a granulomatous PACNS associated with beta-A4 amyloid deposition (the so-called Ab-related angiitis or ABRA)(not shown).
Figure 2.
Figure 2.
Axial T2 w-i (A, B), T1 w-i with contrast enhancement (C, D) show multiple infarcts in thalami and basal ganglia. Note recent infarction with edema and enhancement in the thalamic-capsular region on the right side. Histological examination evidenced a lymphocytic PACNS (see Fig. 4).
Figure 3.
Figure 3.
Granulomatous pattern showing transmural inflammation with prominent mononuclear and granulomatous adventitial inflammation (arrow) (H&E, 200X magnification).
Figure 4.
Figure 4.
Lymphocytic pattern involving intraparenchymal vessels exhibiting perivascular cuffing (A) as well as transmural (B) lymphocytic infiltrates (arrow), with occasional plasma cells (arrowhead) (H&E, 400X magnification).
Figure 5.
Figure 5.
Necrotizing pattern showing transmural fibrinoid necrosis (arrow) with karyorrhectic debris and acute neutrophilic inflammation (H&E. A: 200X magnification, B: 400X magnification).
See this image and copyright information in PMC

References

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