Cristina SartiUniversity of Florence, Italy
Title: Primary Angiitis of the Central Nervous System: a screening algorythm
Primary angiitis of the CNS (PACNS) is a rare and severe disease that can present with variously combined and mainly nonspecific neurological disorders. Neuroradiological features are also not specific. For these reasons, PACNS is a disease that is not only difficult to diagnose but also to suspect. Considering that the confirmation of diagnosis requires invasive investigations (cerebral biopsy/angiography), clinically we felt the need to have a tool to be used for a first level screening of patients to decide which one deserving to be further investigated for PACNS. We sought to create a screening algorithm capable of raising the suspicion of PACNS on the basis of first-level clinical and neuroradiological data. To achieve this, we collected the reported frequencies of clinical and neuroimaging features of PACNS by reviewing the published PACNS case series from 2002 to 2020 and classified them as “major” or “minor” based on their mean value. The characteristics were combined into sets of criteria applied to published PACNS case reports. Those that best identify patients with definite PACNS were selected. 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 and single parenchymal lesion. The selected sets of criteria that could identify all PACNS patients were (1) one clinical (major/minor) + one major neuroradiological feature; and (2) two clinical (? 1 major) + one minor neuroradiological feature. In conclusion, this algorithm is an attempt to help clinicians take the first steps towards diagnosing PACNS. Given the rarity of the disease, we propose a multi-center cooperation for its validation.
Cristina Sarti is a researcher in Neurology, at the University of Florence, Italy. She works at the Stroke Unit of Careggi University Hospital.
Her main clinical and research interest is cerebral ischemia.
- Stroke in the young
- Fabry disease
- Acute stroke mechanism
- Translational research on stroke