Sorleen Trevino1, Justin Salerian1, Akm Muktadir1, Cade Picou2
1Tulane Neurology, 2Tulane University School of Medicine
Background:
Central nervous system vasculopathies encapsulate disorders that result in the inflammatory destruction of blood vessels in the brain, spinal cord, or meninges. We present a unique case of a patient with features of two subtypes co-existing: Inflammatory Cerebral Amyloid Angiopathy (I-CAA) and Primary Angiitis of the Central Nervous System (PACNS).
Results:
An 80-year-old man presented with headaches, worsening memory, disorientation, and lethargy. Initial workup was negative for metabolic derangements, toxins, infections, vitamin deficiencies, systemic inflammation, and seizures. Neurological examination was notable for profound inattention, non-fluent speech, and disorientation. MRI revealed blood products diffusely in the subarachnoid spaces and punctate ischemic strokes not seen on historic scans. Contrasted MRI revealed additional microhemorrhages and ambiguous leptomeningeal enhancement. A lumbar puncture revealed lymphocytic pleocytosis (WBC 41) and a protein elevation of 281, with an elevated IgG Index (1.0) but no oligoclonal bands. He was heterozygous for ApoE ε3/ε4 on genetic testing, and Autoimmune/Paraneoplastic ENC2 and ENS2 panels were negative, along with serum inflammatory, vasculitic, and hypercoagulable studies. Digital subtraction angiography showed no pathologic findings. He subsequently underwent brain and meningeal biopsy, which revealed significant perivascular lymphohistiocytic inflammation over leptomeningeal blood vessels, along with occasional multinucleated giant cells and evidence of fibrinoid necrosis at the vessel wall. APP-positive plaques were seen in the cortex, however, APP was negative in blood vessels. High-dose steroid treatment following his biopsy resulted in a noticeable improvement in attention, memory, and language fluency over the course of a week.
Conclusions:
While biopsy results were inconsistent with I-CAA, his presentation, neuroimaging, and genetic profile could fit this clinical picture. By contrast, the histological features of inflammatory destruction at the blood vessel wall are seen in PACNS. Our case suggests there can be a clinical and pathophysiologic overlap in the phenotypes of I-CAA and PACNS not previously shown in literature.