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Washington University Experience | VASCULAR | Vasculitis - PACNS | 2A0 Case 2 History
Case 2 History ---- The patient was an 80-year-old woman with a history of breast cancer and recent multifocal strokes associated with seizures. She initially presented with right hand numbness, word salad and, later, progressive gait problems and falls. A lumbar puncture showed a monoclonal B-cell lymphocytosis which was also present in the peripheral blood. MRI from 1/27 showed numerous punctate acute infarcts in multiple vascular distributions, leptomeningeal enhancement suspicious for vasculitis and resolving subarachnoid hemorrhage. An angiogram showed mild narrowing of the distal branches above middle cerebral arteries as well as bilateral small aneurysms of the internal carotid arteries at the skull base. Anti-neutrophil cytoplasmic antibodies were negative and her ESR was not elevated. Operative procedure: Left parietal open biopsy. ---- The specimen shows luminal thrombi and fibrinoid necrosis of the vessel walls, which are infiltrated by chronic inflammatory cells including lymphocytes, epithelioid histiocytes and multinucleated giant cells in poorly formed granulomas. Hemosiderin-laden macrophages provide evidence of prior subarachnoid hemorrhage. The parenchyma underlying the leptomeninges shows vasculitis of penetrating vessels which is less severe than in the leptomeninges. Multiple areas of infarction exhibit a central core with tissue destruction, vascular prominence and foamy lipid-laden histiocytes which are surrounded by hyperplastic microglial rod cells and astrogliosis. CD3 immunostain highlights numerous T-cells in the leptomeninges and within the vessel walls, as well as within the parenchyma concentrated around areas of infarction. CD20 highlights significantly fewer B-cells in the leptomeninges with a bland non-neoplastic appearance. Amyloid beta immunohistochemistry shows scattered cored and diffuse plaques in the gray matter, staining nonspecifically the normal structure of vessel elastic lamina but fails to identify intramural deposited beta-amyloid. CD68 identifies granulomas and giant cells within the leptomeninges as well as numerous microglia and foamy histiocytes in infarcted regions. Smooth muscle actin shows fragmentation and destruction of affected leptomeningeal vessels. GMS and AFB are negative for stainable fungal organisms and acid fast bacilli.
