Sarti Cristina, Picchioni Antonella, Telese Roberta, Pasi Marco, Failli Ylenia, Pracucci Giovanni, Cammelli Daniele, Inzitari Domenico
NEUROFARBA Department, Neuroscience Section, University of Florence, Largo Brambilla 3, 50134, Florence, Italy.
Department of Neurosciences, Imaging and Clinical Sciences, University of Chieti-Pescara, Chieti, Italy.
Neurol Sci. 2020 Nov;41(11):3135-3148. doi: 10.1007/s10072-020-04583-3. Epub 2020 Aug 10.
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.
(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.
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.
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.
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.
中枢神经系统原发性血管炎（PACNS）是一种可导致多种神经功能障碍综合出现的病症。其罕见性及千变万化的表现使得诊断甚至怀疑该病都很困难。
（1）对PACNS进行最新综述；（2）基于临床和放射学一级数据创建初步筛查算法，以有助于怀疑PACNS并指导进一步检查。
回顾2002年至2017年发表在PUBMED上的关于PACNS的病例系列，收集临床和神经影像学特征的频率并计算中位数。若频率高于或低于中位数，则将特征分类为“主要”或“次要”。将特征组合成由主要和次要临床及神经放射学特征的所有可能组合所代表的标准集。将标准应用于已发表的PACNS病例报告，并选择最能识别明确PACNS患者的标准。
我们回顾了24个病例系列。“主要”临床特征为头痛、中风、认知障碍、局灶性神经功能缺损；“次要”特征为癫痫发作、意识改变、精神障碍。“主要”神经放射学特征为多个实质病变、实质/脑膜对比增强磁共振血管造影血管异常、血管壁增强；“次要”特征为实质/蛛网膜下腔出血、单个实质病变。能够识别所有PACNS患者的选定标准集为：（1）一项临床（主要/次要）+一项主要神经放射学特征；（2）两项临床（≥1项主要）+一项次要神经放射学特征。
我们的综述提供了PACNS详细的临床/神经放射学情况。所提出的算法应被视为迈向PACNS诊断的初步筛查工具，该诊断需要验证。
