This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.
Abstract
Background: Primary CNS vasculitis (PCNSV) can be diagnosed using cerebral angiography or histopathology combined with clinical features. The original diagnostic criteria, which weigh each test equally, have not been validated. Limited sensitivity and specificity for biopsy and angiography are recognized. We systematically reviewed results of diagnostic tests performed in patients with an ultimate diagnosis of PCNSV.
Methods: We searched the OVID Medline database and bibliographies for original cases of PCNSV. We recorded demographics, diagnostic tests used, and assessed agreement between angiography and biopsy when both tests were performed. We also recorded MRI and CSF results.
Results: We found 701 original cases with PCNSV diagnosed with angiography or pathology. A total of 269 patients (38.4%) had both cerebral angiography and histopathologic testing (biopsy/postmortem). Classic angiographic features of vasculitis were associated with pathologic confirmation in just 32 patients (4.6%). Seventy-four patients (10.6%) with any abnormality on angiography had a normal biopsy, and 99 patients (14.1%) with abnormal biopsies had normal angiography. Brain MRI was abnormal in 505/541 patients (93.3%) and CSF was abnormal in 360/484 patients (74.4%). Increasing use of angiography and decreasing histopathologic testing were found over time.
Conclusions: Cerebral angiography and pathologic tissue examination were undertaken in a minority of published cases with a diagnosis of PCNSV. When both diagnostic tests were performed, disagreement between them was more than 5 times more likely than agreement. Diagnostic criteria for PCNSV may require revision to classify the clinical, pathologic, and radiologic features of this condition more accurately.
Footnotes
Funding information and disclosures are provided at the end of the article. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/cp.
Supplemental data at Neurology.org/cp
- Received December 8, 2016.
- Accepted February 13, 2017.
- © 2017 American Academy of Neurology
AAN Members
We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.
AAN Non-Member Subscribers
Purchase access
The Nerve!: Rapid online correspondence
REQUIREMENTS
If you are uploading a letter concerning an article:
You must have updated your disclosures within six months: http://submit.neurology.org
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.