Angiography‐negative primary central nervous system vasculitis in children: A newly recognized inflammatory central nervous system disease

Abstract

Inflammatory central nervous system (CNS) diseases in childhood comprise a wide spectrum of heterogeneous conditions. We studied 4 children with primary CNS vasculitis in whom results of magnetic resonance imaging studies were abnormal but results of conventional angiography were normal. We determined that angiography-negative, biopsy-confirmed primary smallvessel CNS vasculitis is a previously unrecognized distinct disease entity in children. The diagnosis must be considered in a child with a progressive, acquired diffuse or focal neurologic deficit, even if the results of conventional angiography are normal. A lesional brain biopsy is required to confirm the diagnosis. Use of immunosuppressive therapy plus aspirin leads to an excellent neurologic outcome.

Concordance of Time-of-Flight MRA and Digital Subtraction Angiography in Adult Primary Central Nervous System Vasculitis

“…However, isolated small-sized vessel PCNSV involves a subgroup of patients in whom DSA findings (and a fortiori 3D-TOF-MRA) are also mostly negative, and only biopsy can show vascular involvement in these patients. 9,[20][21][22] In our first description of the French cohort, as well as in another large study by Salvarani et al, 23 twothirds of the patients with biopsy-proved PCNSV had negative DSA findings. 9 In the present study, 6 of our 7 patients with isolated small-sized vessel vasculitis demonstrated by biopsy had normal 3D-TOF-MRA and DSA findings.…”

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].…”

“…This definition excludes small muscular arteries, arterioles, capillaries and venules, which are considered "small vessels of the brain." Therefore, by definition, small vessel cPACNS is an angiography-negative inflammatory brain disease [6]. Within a cerebral vessel, the distinction of proximal segments-defined as the first-and second-order branches-and distal segments-defined as any segments beyond second branching-is commonly made.…”

“…Within a cerebral vessel, the distinction of proximal segments-defined as the first-and second-order branches-and distal segments-defined as any segments beyond second branching-is commonly made. Currently, four distinct subtypes of cPACNS are recognized, two affecting the large-medium vessels and two the small cerebral vessels [5,6]. Nonprogressive cPACNS and progressive cPACNS are subtypes of angiography positive, large-medium cPACNS.…”

“…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].…”