Primary central nervous system vasculitis with prominent leptomeningeal enhancement: A subset with a benign outcome

Abstract

Objective. Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and spinal cord. This study was undertaken to evaluate the clinical features and outcomes among patients with PCNSV who presented with prominent gadolinium meningeal enhancement on magnetic resonance imaging (MRI).Methods. Through retrospective review using the Mayo Clinic medical records linkage system, we identified 101 consecutive patients with PCNSV based on brain biopsy or conventional angiography (or both) between January 1, 1983, and December 31, 2003. We evaluated data on demographics, clinical findings, laboratory studies, imaging, biopsy of brain or spinal cord (or both), treatment, and neurologic outcome.Results. MRIs showed prominent leptomeningeal enhancement in 8 of 101 patients with PCNSV. In 6 of those 8, cerebral angiography or magnetic resonance angiography results were normal, but biopsy of the brain or spinal cord showed vasculitis in all 8. Granulomatous vascular inflammation was found in 6 specimens and was associated in 4 cases with vascular deposits of ␤-amyloid peptide. All 8 patients had a prompt response to therapy, with resolution of the MRI meningeal enhancement. Although 3 of the 8 patients had relapses during followup, the overall outcome was favorable. Patients with meningeal enhancement, compared with patients without enhancement, more commonly had substantial abnormalities of cerebrospinal fluid (100% versus 58%; P ‫؍‬ 0.02) and amyloid angiopathy (50% versus 12%; P ‫؍‬ 0.03).Conclusion. Prominent gadolinium leptomeningeal enhancement on MRI may point to a distinct subtype of PCNSV with small leptomeningeal artery vasculitis and rapid response to therapy.

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.…”

Primary central nervous system vasculitis: clinical experiences with 21 new European cases

“…Since the first histological description in 1922 [5] about 500 cases have been published worldwide [42]. One of the most relevant studies is the recent series of 101 patients from the Mayo Clinic [28,[35][36][37][38][39][40][41].…”

“…Clinically and pathophysiologically it makes sense that patients with microangiopathy have a worse outcome. Further interpretations about outcome concluded on the base of the different subgroups of Mayo Clinic's cohort should be regarded considering potential limitations and bias [28,[35][36][37][38][39][40][41].…”

“…The existing scientific literature on PCNSV is incomplete and difficult to evaluate as much of current understanding comes from smaller case series and single case reports [36].…”

“…Most of the data was assessed retrospective, prospective follow-up was incomplete. It is possible that missing duplication of some characterizations of subgroups described by Salvarini and co-workers is caused by smaller study cohort [28,[35][36][37][38][39][40][41]. The possibility of referral bias has to be calculated.…”