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Washington University Experience | VASCULAR | Vasculitis - PACNS | 10A0 Case 10 History
Case 10 History ---- The patient is a 56-year-old man with a history of hypertension and asthma, who presented with confusion, aphasia, and hallucination in May 2009. He was hospitalized and received an extensive workup, MRI examination (showing diffuse FLAIR changes and meningeal enhancement) and brain biopsy. He was diagnosed as primary CNS vasculitis - giant cell type and was treated with Solu-Medrol 1 gram per day, for five days, followed by prednisone and cyclophosphamide by mouth every day. Afterwards, the patient's symptoms much improved. ---- Repeated brain MRI a year later showed Interval resolution of leptomeningeal enhancement and associated increased FLAIR signal. ---- The brain biopsy consists of dura, cortex and underlying white matter. Within the leptomeninges and cerebral cortex, many of the small to medium sized vessels show features of vasculitis, including the presence of perivascular and intravascular lymphocytes, macrophages, and multinucleated giant cells. No well-developed granulomas are seen although there are scattered epithelial histiocytes and multinucleate giant cells. Some of the vessels display foci of apparent fibrinoid necrosis. A CD68 stain highlights numerous macrophages and multinucleated giant cells in the perivascular spaces. Scattered activated microglial cells are also highlighted in the underlying cerebral cortex.
