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Washington University Experience | VASCULAR | Vasculitis - PACNS | 9A0 Case 9 History
Case 9 History ---- The patient is a 47 year old woman who suffered initial onset of generalized tonic-clonic seizure on 05/28 which was preceded by an intense deja vu. She has had multiple episodes of persistent deja vu in the past without a seizure of any motor type. She has no other complaints except for intermittent nausea and a history of headaches. There is no history of head trauma. Her past medical history is significant only for asthma and borderline hypertension. Workup demonstrated a right temporal lobe mass. Operative procedure: Craniotomy ---- Sections show granulomatous inflammation. The granulomas percolate along the Virchow-Robin spaces and embrace blood vessels, usually small venules and capillaries; however, there is little evidence of frank angionecrosis and large arterial vessels are not included in the biopsy. The adjacent brain parenchyma shows reactive astrocytosis, although not microinfarction. Some vessels show perivascular lymphocytic infiltration without epithelioid histiocytes. Special stains, including AFB and GMS are negative. A Verhoeff Von Gieson stain is non-contributive. An immunohistochemical stain for smooth muscle actin shows little vascular destruction, although a few vessels appear to have disrupted SMA staining which may reflect the passage of hematologic reactive elements into the brain parenchyma. ---- The differential diagnosis included sarcoidosis and primary angiitis of the CNS. There is no history of systemic angiitis, infection or sarcoidosis, the latter a diagnosis of exclusion and clinicopathologic correlation. The profound angiocentricity of the lesions strongly suggests the diagnosis of PACNS. Our experience includes a number of cases of granulomatous angiitis in which frank angionecrosis has not been demonstrated. The sparsity of subarachnoid infiltrate suggests apparent angiocentricity does not reflect involvement of the subarachnoid space and simple propagation into the brain of a leptomeningeal infiltrate.
