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Primary CNS vasculitis with spinal cord involvement

From Unità Operativa di Reumatologia (C.S.), Arcispedale S. Maria Nuova, Reggio Emilia, Italy; Department of Neurology (C.S. [visiting physician], R.D.B.), Division of Biostatistics (T.J.H.C.), Department of Radiology (J.H.), Division of Anatomic Pathology (C.G., D.V.M.), and Division of Rheumatology (G.G.H. [Emeritus Member]), Mayo Clinic, Rochester, MN; and Division of Rheumatology (K.T.C.) and Department of Neurology (J.F.M.), Mayo Clinic, Jacksonville, FL.

Address correspondence and reprint requests to Dr. Robert D. Brown, Jr., Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905 brown{at}mayo.edu

Background: Primary CNS vasculitis (PCNSV) is an uncommon disease in which lesions are limited to the brain and spinal cord. Our objective was to evaluate the frequency, clinical features, and outcome of spinal cord involvement in PCNSV.

Methods: We retrospectively identified 101 consecutive patients with PCNSV. Spinal cord involvement was documented for five. Clinical findings, laboratory studies, and outcomes of patients with spinal cord involvement were assessed and compared with those without spinal cord manifestations.

Results: Spinal cord symptoms developed before cerebral symptoms in one patient, concurrently in two, and after cerebral symptoms in two. CNS biopsy specimens showed necrotizing vasculitis in three patients and granulomatous vasculitis in two. MRI of the spinal cord showed enhanced thoracic lesions in all five. Cerebral angiograms from four patients had normal findings. One patient had a fatal clinical course. The other four had relapses during follow-up but responded well to therapy and had favorable overall outcomes. At the last follow-up (median, 19 months after diagnosis), the four patients had recovered with slight or moderate residual disability. No significant differences in clinical and laboratory features were observed when comparing patients with or without spinal cord involvement. Cerebral angiograms with evidence of vasculitis were significantly more frequent for patients without spinal cord involvement (p = 0.002).

Conclusion: Spinal cord involvement was documented in 5% of patients with primary CNS vasculitis. The thoracic cord was the predominantly affected site. Other than myelopathy, clinical characteristics were similar to those of the patients without spinal cord involvement.

GLOSSARY: ATM = acute transverse myelitis; ESR = erythrocyte sedimentation rate; ND = no data; PCNSV = primary CNS vasculitis; WBC = white blood cells.

Supplemental data at www.neurology.org

Disclosure: The authors report no disclosures.

Received June 19, 2007. Accepted in final form February 22, 2008.

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				CNS Vasculitis with Spinal-Cord Involvement Journal Watch Neurology, September 9, 2008; 2008(909): 3 - 3. [Full Text]