Primary CNS vasculitis with spinal cord involvement.

C. Salvarani, Robert D Jr. Brown, K. T. Calamia, T. J. Christianson, John III Huston, James F Meschia, Caterina Giannini, D. V. Miller, G. G. Hunder

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Abstract

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.

Original languageEnglish (US)
Pages (from-to)2394-2400
Number of pages7
JournalNeurology
Volume70
Issue number24 Pt 2
StatePublished - Jun 10 2008
Externally publishedYes

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Central Nervous System Vasculitis
Spinal Cord
Vasculitis
Angiography
Spinal Cord Diseases

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Salvarani, C., Brown, R. D. J., Calamia, K. T., Christianson, T. J., Huston, J. III., Meschia, J. F., ... Hunder, G. G. (2008). Primary CNS vasculitis with spinal cord involvement. Neurology, 70(24 Pt 2), 2394-2400.

Primary CNS vasculitis with spinal cord involvement. / Salvarani, C.; Brown, Robert D Jr.; Calamia, K. T.; Christianson, T. J.; Huston, John III; Meschia, James F; Giannini, Caterina; Miller, D. V.; Hunder, G. G.

In: Neurology, Vol. 70, No. 24 Pt 2, 10.06.2008, p. 2394-2400.

Research output: Contribution to journalArticle

Salvarani, C, Brown, RDJ, Calamia, KT, Christianson, TJ, Huston, JIII, Meschia, JF, Giannini, C, Miller, DV & Hunder, GG 2008, 'Primary CNS vasculitis with spinal cord involvement.', Neurology, vol. 70, no. 24 Pt 2, pp. 2394-2400.
Salvarani C, Brown RDJ, Calamia KT, Christianson TJ, Huston JIII, Meschia JF et al. Primary CNS vasculitis with spinal cord involvement. Neurology. 2008 Jun 10;70(24 Pt 2):2394-2400.
Salvarani, C. ; Brown, Robert D Jr. ; Calamia, K. T. ; Christianson, T. J. ; Huston, John III ; Meschia, James F ; Giannini, Caterina ; Miller, D. V. ; Hunder, G. G. / Primary CNS vasculitis with spinal cord involvement. In: Neurology. 2008 ; Vol. 70, No. 24 Pt 2. pp. 2394-2400.
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AU - Meschia, James F

AU - Giannini, Caterina

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N2 - 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.

AB - 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.

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