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Spinal Cord Involvement in Primary Angiitis of the Central Nervous System: A Report of Two Cases

Adriana CampiGo,a, G. Benndorfa, V. Martinellia, M. R. Terrenia and G. Scottia

a From the Departments of Neuroradiology (A.C., G.S.), Neurology (V.M.), and Pathology (M.R.T.), Scientific Institute and University San Raffaele, Milan, Italy; and the Department of Radiology, Charité, Humboldt University Berlin, Germany (G.B.).



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FIG 1. Patient 1: 29-year-old woman with primary angiitis of the CNS whose progressive paraparesis started 1 month after the onset of cerebral symptoms.

A–F, First MR study. Sagittal FSE T2-weighted (4700/112) images (A–C) show diffuse increased signal intensity within the cervical cord (arrows, A). Skip areas of slightly increased signal intensity are detectable within the upper and lower thoracic cord (arrows, B and C). Sagittal contrast-enhanced SE T1-weighted (500/12) images (D and E) show multiple small homogeneously enhancing areas of the cervical and thoracic spinal cord (arrows), primarily posterior in location, and pial enhancement of the conus. Axial contrast-enhanced SE T1-weighted (500/15) image (F) shows two posteriorly located punctate areas of homogeneous enhancement (arrows).



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FIG. 1. Continued.

G–I, Follow-up MR study 12 months later. Sagittal FSE T2-weighted (4700/112) images (G and H) show regression of the increased signal intensity within the entire cord. Sagittal contrast-enhanced SE T1-weighted (500/12) image (I) shows no evidence of contrast enhancement of the cervico-thoracic cord.

J and K, Microscopic sections show a small cortical vessel with its wall infiltrated by a proliferation of mononuclear cells in rarefied gliotic cerebral cortex (J) and fibrinoid necrosis with nuclear and cytoplasmic debris (arrows, K) and loss of normal structure of the white matter (K) (hematoxylin-eosin, original magnification x275).



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FIG 2. Patient 2: 50-year-old man with primary angiitis of the CNS who presented with progressive paraparesis.

A and B, First MR study, obtained at 0.5 T. Sagittal contrast-enhanced SE T1-weighted images show numerous small homogeneously enhancing lesions located in the cervical and thoracic cord (arrow, A; arrowheads, B). Enhancing lesions can also be seen in the medulla, both dorsally and ventrally.

C and D, Follow-up MR study 36 months after the onset of disease. Sagittal contrast-enhanced SE T1-weighted (500/12) images show no evidence of contrast enhancement of the cord.