Brown, Robert D

BACKGROUND: Spinal cord involvement is infrequently reported in primary central nervous system vasculitis (PCNSV). This study describes the clinical findings, therapeutic responses, and outcomes of spinal cord involvement in a large cohort of patients with PCNSV. METHODS: We retrospectively studied 216 consecutive patients with PCNSV evaluated at the Mayo Clinic from 1983 to 2023. Spinal cord involvement was documented by histological examination or imaging. The clinical, laboratory, radiological, and pathological findings, along with management and outcomes, were compared between patients with and without spinal cord involvement. RESULTS: Of 216 patients, 10 (4.6%) had spinal cord involvement, with cerebral involvement in 9 cases. One patient (0.5%) had isolated spinal cord vasculitis. Histological evidence of vasculitis was found in all 10, with necrotizing vasculitis in 5 (50%). Magnetic resonance imaging showed thoracic abnormalities in 8 patients, cervical spine involvement in 2, conus medullaris involvement in 3, and cauda equina enhancement in 4. Compared with the 206 patients without spinal cord involvement, those with spinal cord vasculitis were more likely to present with paraparesis/tetraparesis ( P <0.001) and necrotizing vasculitis ( P =0.01) and less likely with hemiparesis ( P =0.006) and granulomatous vasculitis ( P =0.03). Patients with spinal cord involvement were more likely to have at least 1 relapse ( P <0.001) or more ( P <0.001). No differences between the 2 groups were observed regarding long-term remission, therapy response, and high disability scores (modified Rankin Scale score, 4–6) or death at last follow-up. Spinal cord involvement was associated with meningeal enhancement on brain imaging (odds ratio, 10.50) and the presence of lymphoma (odds ratio, 6.49), specifically Hodgkin lymphoma diagnosed simultaneously with PCNSV. Spinal involvement was negatively associated with increasing age (odds ratio, 0.64) and cerebral infarction on imaging (odds ratio, 0.08). CONCLUSIONS: Spinal cord involvement defines a distinct subset of patients with PCNSV.

BACKGROUND: Cervical artery dissection (CeAD) represents up to 15% to 25% of ischemic strokes in people under the age of 50 years. Noninvasive vessel imaging is increasingly used in clinical practice, but the impact on the frequency of detection of CeAD is unknown. In 2006, the yearly incidence rate of CeAD was estimated at 2.6 per 100 000 person-years, but the current incidence is unknown. METHODS: In this population-based retrospective observational cohort study, we utilized the resources of the Rochester Epidemiology Project to ascertain all adult residents of Olmsted County, MN, diagnosed with internal carotid artery dissection and common carotid artery dissection or vertebral artery dissection from 2002 to 2020. Patients with only intracranial involvement or CeAD following major trauma were excluded. Age-adjusted sex-specific and age- and sex-adjusted incidence rates were estimated using the US White 2010 decennial census, with rates expressed per 100 000 person-years. We assessed longitudinal trends by dividing the data into 5-year time intervals, with the last being a 4-year interval. RESULTS: We identified 123 patients with a diagnosis of CeAD. There were 63 patients with internal carotid artery dissection, 54 with vertebral artery dissection, 2 with concurrent internal carotid artery dissection and vertebral artery dissection, and 4 with common carotid artery dissection. There were 63 (51.2%) female patients and 60 (48.8%) male patients. The average age at diagnosis was 50.2 years (SD, 15.1 [95% CI, 20.1–90.5] years). The incidence rate of spontaneous CeAD encompassing all locations was 4.69 per 100 000 person-years (2.43 for internal carotid artery dissection and 2.01 for vertebral artery dissection). The incidence rate increased from 2.30 per 100 000 person-years from 2002 to 2006 to 8.93 per 100 000 person-years from 2017 to 2020 ( P <0.0001). The incidence rate for female patients rose from 0.81 per 100 000 person-years from 2002 to 2006 to 10.17 per 100 000 person-years from 2017 to 2020. CONCLUSIONS: The incidence rate of spontaneous CeAD increased nearly 4-fold over a 19-year period from 2002 to 2020. The incidence rate in women rose over 12-fold. The increase in incidence rates likely reflects the increased use of noninvasive vascular imaging.