Oh Oh Oh It's Magic, You Know…Never Believe It's Not So! (Slides)

A biopsy of the right temporal lobe showed chronic inflammation with no granulomas or organisms present. A PET scan demonstrated focal hypermetabolism in the region of the left parotid gland. A fine needle aspirate revealed the parotid gland lesion to be a Warthin tumor. Anti-fungal therapy was broadened and albendazole was initiated along with dexamethasone. The CSF profile improved with a decrease in protein, opening pressure and eosinophilia - however, the neurologic exam remained unchanged. The bilateral optic disc swelling worsened and new peripapillary splinter hemorrhages developed every two to three days. Repeat MRI revealed yet more areas of new infarction and leptomeningeal enhancement. A biopsy of the right occipital lobe was performed. The pathology demonstrated markedly thickened vessel walls due to accumulation of homogenous eosinophilic material, perivascular granulomatous and transmural lymphoplasmacytic inflammation with several foci of vessel wall destruction, diagnostic of vasculitis. In addition, there were areas of ischemic damage with collections of foamy macrophages, reactive gliosis, and tissue rarefaction consistent with subacute infarcts. Congo red stain highlighted abundant amyloid within the majority of blood vessel walls. Immunohistochemistry for beta amyloid was extensively positive in blood vessels. The features were consistent with amyloid beta-related angiitis (ABRA) associated with secondary subacute cerebral infarcts. All anti-pathogen treatments were discontinued and intravenous high-dose methylprednisolone and cyclophosphamide were initiated. The optic nerve head findings resolved and the opening pressure normalized. Unfortunately, the patient remained severely encephalopathic with significant left-sided focal neurologic deficits at his last evaluation.