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Washington University Experience | VASCULAR | Vasculitis - PACNS | 15A0 Case 15 History
Case 15 History ---- The patient is a 40-year-old man with lupus and recent encephalopathy on warfarin (due to mitral valve replacement). MRI from 7/23 showed pontine hemorrhage, extensive vasogenic edema of the medial temporal lobes, inferior frontal lobes and mesencephalon, and enhancement of the basilar leptomeninges. The radiographic differential diagnosis included rhombencephalitis, vasculitis, neuro-Behçet's, neurosarcoidosis, or neoplasm such as lymphoma or glioma. Operative procedure: Open brain biopsy. ---- The microvasculature and arterioles up to ~1 mm diameter display abundant perivascular and transmural active and mixed chronic inflammation, including polymorphonuclear leukocytes, lymphocytes, plasma cells, and macrophages. Most involved vessels show frank mural angionecrosis, frequently with fibrin deposition. Perivascular hemosiderin deposition indicates prior microhemorrhage. The white matter is reactive and exhibits astrocytosis. CD3 highlights abundant perivascular and intramural T-cells. Smooth muscle actin demonstrates focal disruption in vessels damaged by inflammation. Trichrome stain shows destruction of the fibroconnective vascular matrix. Beta-amyloid immunostain shows no plaques and no vascular amyloid deposition. A thioflavin S stain for amyloid is also negative. In this case the presence of SLE with its known vasculopathy would not represent a typical form of PACNS.
