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Review
. 2020 Nov;41(11):3135-3148.
doi: 10.1007/s10072-020-04583-3. Epub 2020 Aug 10.

"When should primary angiitis of the central nervous system (PACNS) be suspected?": literature review and proposal of a preliminary screening algorithm

Cristina Sarti  1 , Antonella Picchioni  2 , Roberta Telese  3 , Marco Pasi  4 , Ylenia Failli  2 , Giovanni Pracucci  2 , Daniele Cammelli  5 , Domenico Inzitari  2
Affiliations
Review

"When should primary angiitis of the central nervous system (PACNS) be suspected?": literature review and proposal of a preliminary screening algorithm

Cristina Sarti et al. Neurol Sci. 2020 Nov.

Erratum in

Abstract

Background: Primary angiitis of the CNS (PACNS) is a process causing variously combined neurological disturbances. Its rarity and kaleidoscopic presentation make it difficult to diagnose and even to suspect.

Objective: (1) To provide an up-to-date review on PACNS and (2) to create a preliminary screening algorithm based on clinical and radiological first-level data, useful to suspect PACNS and guide further investigations.

Methods: Review of PUBMED case series on PACNS, published from 2002 to 2017, collection of frequencies of clinical and neuroimaging features and calculation of median values. Classification of features as "major" or "minor" if frequency was higher or lower than median value. Combination of features in sets of criteria represented by all possible combinations of major and minor clinical and neuroradiological features. Application of criteria to published PACNS case reports and selection of the ones best identifying patients with definite PACNS.

Results: We reviewed 24 case series. "Major" clinical features were headache, stroke, cognitive impairment, focal neurological deficits; "minor" were seizures, altered consciousness, psychiatric disorders. "Major" neuroradiological features were multiple parenchymal lesions, parenchymal/meningeal contrast enhancement, magnetic resonance angiography vessel abnormalities, vessel wall enhancement; "minor" were parenchymal/subarachnoid hemorrhage, single parenchymal lesion. The selected sets of criteria able to identify all PACNS patients were (1) one clinical (major/minor) + one major neuroradiological feature; and (2) Two clinical (≥ 1 major) + one minor neuroradiological feature.

Conclusion: Our review provides a detailed clinical/neuroradiological picture of PACNS. The proposed algorithm should be regarded as a preliminary screening tool to move the first steps towards PACNS diagnosis that needs validation.

Keywords: CNS vasculitis; Cerebral angiography; Magnetic resonance imaging; PACNS; Primary angiitis of the CNS.

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Conflict of interest statement

All authors declare that they have no conflict of interest.

Figures

Fig. 1
Fig. 1
Clinico-radiological screening algorithm
See this image and copyright information in PMC

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