Ataxia at the Masquerade Ball

MRI from 2007 and 2012 were re-reviewed and multiple punctate hyperintense foci were identified that had not been previously appreciated. These foci had increased in number on repeat imaging in 2013. Brain biopsy May 2013 revealed multifocal perivascular and intramural chronic inflammatory cells, with focal astrogliosis and neither granulomas nor giant cells. Leptomeningeal biopsy showed fibrotic leptomeninges with hyperplastic meningothelial cells and psammoma body deposition. Immunohistochemistry was negative for CMV, VZV, congo red, and thioflavin. Immunohistochemical staining also showed predominantly CD3-positive T-cells. Cerebral angiogram showed irregularity of right and left internal carotid arteries and right vertebral artery suggestive of vasculitis. He was treated with steroids with complete resolution of the enhancing lesions on repeat brain MRI. His clinical picture has improved slightly on chronic immunosuppression. Primary CNS vasculitis is rare, with non-specific signs and symptoms of CNS dysfunction. It is regarded as a diagnosis of exclusion. Three histopathologic presentations are recognized: granulomatous, lymphocytic, and necrotizing. There are no validated diagnostic criteria for primary CNS vasculitis and although angiography is helpful, it has limited sensitivity. Brain biopsy has strong negative and positive predictive values. A Mayo Series followed 101 patients for an average of 13 months and found that 81% responded favorably to prednisone. Of these patients, there was increased mortality despite treatment and approximately one quarter had relapse of their illness. Pizzanelli et al. reported a series of 8 patients. Patients classified as having moderate disease were treated with steroids. Patients with severe disease were treated with steroids plus immunosuppression. Patients were followed for 7-62 months with only one patient suffering a relapse. They concluded that brain biopsy and angiography are helpful for diagnosis and that treatment should be tailored to the patient's clinical picture until diagnostic and therapeutic guidelines are developed.