Abstract
BackgroundPrimary 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.MethodsReview 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.ResultsWe 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.ConclusionOur 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.
Highlights
Primary angiitis of the central nervous system (PACNS) is a rare form of vasculitis affecting only CNS blood vessels, with no evidence of vasculitis in other organs and systems [1]
Patients with PACNS can present with non-specific neurological disorders and symptoms, such as ischemic or hemorrhagic stroke, cognitive impairment, headache, seizures, and psychiatric disorders [1]
We found 24 case series [1, 3, 5–28], amounting to a total of 585 patients with a diagnosis of PACNS, 41% biopsy proven
Summary
Primary angiitis of the central nervous system (PACNS) is a rare form of vasculitis affecting only CNS blood vessels, with no evidence of vasculitis in other organs and systems [1]. Recognized for the first time in the 1950s [1], it has been poorly described until 1988, when Calabrese and Mallek proposed their diagnostic criteria [2], requiring the presence of an otherwise unexplained neurological or psychiatric deficit, the presence of either classic angiographic or histopathological features of angiitis of the CNS, and no evidence of systemic vasculitis or any other disorder that could cause or mimic the angiographic or pathological features of the disease. PACNS can progress, leading to moderate-severe disability or even death, while, if recognized and treated early, complete recovery may occur. For this reason, early diagnosis and treatment are mandatory [4]. 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
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