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ESOC 2026 | Current guidelines for the diagnosis and treatment of PACNS

Marialuisa Zedde, MD, AUSL Reggio Emilia, Reggio Emilia, Italy, discusses the current guidelines for diagnosing and treating primary angiitis of the central nervous system (PACNS), highlighting the limitations of existing literature due to inconsistent population studies and underreported or variably described neurological and neuroradiological findings. This interview took place at the 12th European Stroke Organisation Conference (ESOC) in Maastricht, The Netherlands.

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Transcript

We performed a great effort with ESO three years ago with a dedicated committee about the first guidelines about diagnosis and treatment of PACNS. So the most relevant finding in this work was that many of the well-known and cited literature papers are not perfectly done because the population studies are different and differently described, article by article, and even the most common neurological findings and neuroradiological findings are underreported or reported with different words...

We performed a great effort with ESO three years ago with a dedicated committee about the first guidelines about diagnosis and treatment of PACNS. So the most relevant finding in this work was that many of the well-known and cited literature papers are not perfectly done because the population studies are different and differently described, article by article, and even the most common neurological findings and neuroradiological findings are underreported or reported with different words. For example, we don’t have any information about how many patients underwent post-contrast MRI sequence, what sequence, and how many patients had an acute stroke, and what is acute stroke in these settings. So we have also an overconfidence about interpretation of CSF findings, but the so-called inflammatory profile in CSF is totally nonspecific, nor for PACNS, neither for any other vascular disease or non-vascular disease in the brain. So one out of four patients with PACNS have normal CSF findings. This is because the diagnostic criteria of PACNS are not really diagnostic criteria as we consider, because they have no validation, they are not sensitive or specific, and we cannot use them as we use the diagnostic criteria for other more frequent diseases, for example.

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