Primary angiitis of the central nervous system: emerging variants

Abstract

Similarities between SVD PACNS and microscopic polyarteritis suggest the former may represent a limited form of the latter. MVD PACNS has a distinctly more benign relapse pattern than its multisystem counterpart polyarteritis nodosa. Acute-phase serology was useful in designating inflammatory processes at presentation of patients presenting with encephalopathy caused by SVD only, but were unhelpful in defining relapses in this form of PACNS, the definition of which in all cases rested on clinical assessment and MR scanning. Direct cerebral angiography was not diagnostic in any case of SVD PACNS; positive brain biopsy is diagnostically unequivocal, but the total clinical syndrome with imaging may establish a diagnosis with highest probability. In MVD PACNS, angiography with MR scan proved diagnostic. We suggest an algorithm for a rational, minimally invasive approach to investigation. In PACNS, SVD and MVD are important variants, and decisions about therapy should incorporate these distinctions.

Primary central nervous system vasculitis: analysis of 101 patients

“…However, in the group of 70 patients with diagnoses established by angiography, relapses were more frequent for those with large-vessel involvement (30%) than those with only small-vessel changes (9%). These findings differed from MacLaren and colleagues' 28 findings, which noted more frequent relapses for patients with small-vessel involvement. Differences in the definition of "large artery" may explain this disparity.…”

“…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.…”

Original article Primary angiitis of the central nervous system: a study of histopathological patterns and review of the literature

“…Amongst patients attending the regional vasculitis clinic at Manchester Royal Infirmary between 1988 and 2003 (n = 105), the frequency of PACNS was 10.5% [21]. The frequency of PACNS amongst vasculitides and connective tissue diseases associated with a diagnosis of CNS …”

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

“…For DSA and 3D-TOF-MRA analysis, in line with previous works, we differentiated large-, medium-, and small-sized vessels. 11,12 Intracranial internal carotid and proximal anterior (A1), middle (M1), and posterior (P1) cerebral arteries were considered large; second divisions (A2, M2, P2) and subsequent branches (ϾA2, ϾM2, ϾP2) were considered medium-and small-sized vessels, respectively. The vertebral and basilar arteries were considered largesized vessels, whereas the PICA, anterior inferior cerebellar artery, and SCA were considered medium-sized vessels.…”