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Washington University Experience | VASCULAR | Vasculitis - PACNS | 6A0 Case 6 History
Case 6 History ---- The patient is a 29 year old man with a history of treated Rocky Mountain spotted fever one year previously, who recently presented with altered mental status and a possible seizure. CSF showed an increased number of nucleated cells. Serum and CSF tests for viral, fungal and bacterial pathogens were negative. MRI on 9/12 showed leptomeningeal enhancement on the posterior cerebellar surface. Whole-body positron emission tomography (PET) scan on 9/13 showed small subcarinal nodes with calcific foci and no increased FDG uptake; interpreted as consistent with old granulomatous lung disease. No metabolically active disease indicative of malignancy or infection was seen systemically. A PPD skin test was negative. Operative procedure: Right sided posterior fossa craniotomy for open brain biopsy. ---- The neurosurgical specimen showed diffuse involvement of the leptomeninges by non-necrotizing granulomatous inflammation which tracked along the Virchow-Robin spaces into the parenchyma. Many parenchymal granulomas are perivascular. While some granulomas appear 'naked', a few have lymphocytic cuffing. Histochemical stains (PAS-D, Gram, GMS and AFB) are negative for microorganisms. Although these features are suggestive of primary CNS angiitis, the patient’s young age as well as presence of calcified subcarinal lymph nodes is perplexing. Further clinical correlation was recommended to rule out sarcoidosis and/or systemic vasculitis.
