RT Journal Article
SR Electronic
T1 The diagnosis of primary central nervous system vasculitis
JF Practical Neurology
JO Pract Neurol
FD BMJ Publishing Group Ltd
SP 109
OP 114
DO 10.1136/practneurol-2018-002002
VO 20
IS 2
A1 Claire M Rice
A1 Neil J Scolding
YR 2020
UL http://pn.bmj.com/content/20/2/109.abstract
AB The diagnosis of primary central nervous system (CNS) vasculitis is often difficult. There are neither specific clinical features nor a classical clinical course, and no blood or imaging investigations that can confirm the diagnosis. Contrast catheter cerebral angiography is neither specific nor sensitive, yet still underpins the diagnosis in many published studies. Here we describe an approach to its diagnosis, emphasising the importance of obtaining tissue, and present for discussion a new, binary set of diagnostic criteria, dividing cases into only ‘definite’ primary CNS vasculitis, where tissue proof is available, and ‘possible,’ where it is not. We hope that these criteria will be modified and improved by discussion among experts, and that these (improved) criteria may then be adopted and used as the basis for future prospective studies of the clinical features and diagnosis of this difficult and dangerous disorder, particularly for coordinated multicentre therapeutic trials.