Primary central nervous system vasculitis in children

Abstract

Objective. Primary angiitis of the central nervous system (PACNS) is a severe and ill-defined neurologic disease. The goal of this study was to characterize the presenting features, treatment, and neurologic outcome of PACNS in children (cPACNS) and to define the predictors of disease progression in order to identify high-risk patients with cPACNS.Methods. The cohort comprised consecutive patients diagnosed as having cPACNS based on clinical and vascular imaging findings, including identification of arterial stenosis on conventional angiography or magnetic resonance (MR) angiography. Disease progression was defined angiographically at >3 months after initial angiography. Clinical data obtained in prospectively collected standardized assessments and results of laboratory tests, including detection of cerebrospinal fluid abnormalities, were noted, and neuroimaging studies were reanalyzed. Predictors of progression were identified and tested in multivariate regression models.Results. Sixty-two consecutive patients with cPACNS (38 male, 24 female, median age 7.2 years) were included. Two distinct subgroups were identified, those with progressive disease and those with nonprogressive disease. Progressive cPACNS was found in 20 of 62 children and was predicted by a clinical presentation of neurocognitive dysfunction, multifocal parenchymal lesions on MR imaging, and evidence of distal stenoses on angiography.Conclusion. The spectrum of PACNS in children includes both progressive and nonprogressive forms. Characteristic features at diagnosis can be used to predict later progression, to identify a distinct high-risk cPACNS cohort, and to help guide selection of patients for immunosuppressive therapy.

Primary central nervous system vasculitis: analysis of 101 patients

“…In contrast with our histological criteria, criteria used in some previous studies did not require transmural inflammation; other studies included varicella zoster infection and abnormal findings from conventional angiography and MRA that were frequently unilateral or limited to a single vessel. 28,31,32 Clinical findings in our series that were similar to those of previous reports included the sex distribution, median age of onset in early middle age, and the broad spectrum of clinical manifestations. Most laboratory test results were also similar to those of earlier studies.…”

“…Furthermore, in the largest study of PCNSV in children, diagnoses for all were established by conventional angiography or MRA with frequent focal or unilateral changes. 31 Our diagnostic criteria were more conservative than some earlier investigations. As a result, more than 90% of our conventional angiograms with positive findings showed multiple bilateral vessel alterations of vasculitis.…”

Treatment of CNS Vasculitis in Children

“…Distinct subtypes have been identified based on the vessel size involved and course of inflammatory illness [5,6]. Largemedium vessel cPACNS or angiography-positive cPACNS is an inflammatory vascular disease that can affect the intracranial internal carotid arteries (ICA), the circle of Willis and its branches, the posterior cerebral circulation, and the spinal vessels up to the resolution of 3-T magnetic resonance angiography and/or conventional angiography [5,9].…”

“…In 1988, Calabrese [1] proposed diagnostic criteria for adults with PACNS including the following: newly acquired focal or diffuse neurological deficits plus angiographic or histological evidence of CNS vasculitis in the absence of a systemic condition that could explain or mimic these findings [1]. Over the past decade, these were modified and adopted for childhood PACNS (cPACNS) [5,6].…”

Rituximab for Primary Angiitis of the Central Nervous System: Report of 2 Patients from the French COVAC Cohort and Review of the Literature

“…Only a positive biopsy can ascertain the diagnosis, and all appropriate staining, immunohistochemistry studies, and immunophenotyping are needed to rule out related conditions such as amyloid-β-related angiitis or different mimicking diseases such as CNS lymphoma. However, in practice and for more than two-thirds of patients in previously referenced series, biopsy was not performed and diagnosis relied on the combination of vascular changes on CCA and the exclusion, after thorough clinical and laboratory evaluation, of other conditions that can involve brain vessels 1,5,6,7,8 . Our 2 patients underwent extensive investigations to rule out differential diagnoses.…”