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Published on: September 18, 2011
PCNSV With Spinal Cord Involvement: A 40-Year Single-Center Study.
Carlo Salvarani1,2, Robert D Brown1, Teresa J H Christianson3
1Department of Neurology (C.S., R.D.B.), Mayo Clinic, Rochester, MN.
Spinal cord vasculitis is rare in primary central nervous system vasculitis (PCNSV) but presents distinct clinical features. Patients with spinal cord involvement show higher relapse rates but similar long-term outcomes compared to those without.
Area of Science:
- Neurology
- Immunology
- Vascular Medicine
Background:
- Primary central nervous system vasculitis (PCNSV) rarely involves the spinal cord.
- Understanding the characteristics of spinal cord involvement in PCNSV is crucial for diagnosis and management.
- This study aims to delineate the clinical profile, treatment, and outcomes of PCNSV with spinal cord lesions.
Purpose of the Study:
- To describe the clinical findings, therapeutic responses, and outcomes of spinal cord involvement in a large cohort of patients with PCNSV.
- To compare patients with and without spinal cord involvement to identify distinct features and associations.
Main Methods:
- Retrospective analysis of 216 consecutive PCNSV patients evaluated at Mayo Clinic (1983-2023).
- Spinal cord involvement confirmed by histology or imaging.
- Comparison of clinical, radiological, pathological, and outcome data between patients with and without spinal cord involvement.
Main Results:
- 10 patients (4.6%) had spinal cord involvement, mostly with concurrent cerebral involvement.
- Spinal cord vasculitis patients were more likely to present with paraparesis/tetraparesis and necrotizing vasculitis, and less likely with hemiparesis or granulomatous vasculitis.
- Spinal cord involvement was associated with higher relapse rates, meningeal enhancement, and lymphoma (especially Hodgkin lymphoma), but not with long-term disability or mortality.
Conclusions:
- Spinal cord involvement represents a distinct subset of PCNSV patients.
- Early identification and tailored management are important given the higher relapse risk.
- Association with lymphoma warrants further investigation.
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