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Presentation transcript:

Neurology Resident and Fellow Section Cerebral Amyloid Angiopathy-Related Inflammation Presenting with Isolated Leptomeningitis Teaching NeuroImages Neurology Resident and Fellow Section © 2017 American Academy of Neurology

Vignette A 69-year-old woman presented with one-month of rapid cognitive decline preceded by one year of very mild memory changes. Kang et al. © 2017 American Academy of Neurology

Imaging Kang et al. Figure 1. Magnetic resonance brain imaging. T1-weighted images post-gadolinium (A) demonstrate leptomeningeal enhancement of the bilateral temporal, parietal, and occipital lobes (arrowheads), with associated non-suppression of sulcal cerebrospinal fluid (arrows) on fluid attenuated inversion recovery (B). No microhemorrhages or areas of restricted diffusion were seen on gradient-echo or diffusion weighted images (not shown). Kang et al. © 2017 American Academy of Neurology

Imaging Figure 2. A) Vascular amyloid deposition with perivascular inflammation but no overt angiitis. Parenchyma shows reactive gliosis. B) Beta-amyloid immunostain demonstrating amyloid angiopathy (arrows) and plaque (arrowhead). C) CD3 and CD68 immunostains show no definite angioinvasion by immune cells. Kang et al. © 2017 American Academy of Neurology

Cerebral Amyloid Angiopathy-Related Inflammation Presenting with Isolated Leptomeningitis Examination showed impaired attention, logopenic aphasia, anosagnosia, simultagnosia, graphesthesia, and apraxia. Neuroimaging demonstrated leptomeningeal enhancement without significant parenchymal lesions or cerebral microbleeds (figure 1). A lymphocytic pleocytosis (15 nucleated cells/μl) was noted on cerebrospinal fluid analysis. Extensive diagnostic testing was inconclusive, including flow cytometry and cytology, conventional angiography, body computed tomography and positron emission tomography. Cerebral amyloid angiopathy-related inflammation (CAA-RI) was confirmed on brain biopsy (figure 2). CAA-RI rarely presents with isolated leptomeningeal enhancement and can be a challenging diagnosis (1, 2). Kang et al. © 2017 American Academy of Neurology