Intravascular Lymphoma Involving the CNS: a case report underscoring the importance of brain biopsy for progressive cerebrovascular disease (P6.095)

Objective: N/A Background: Intravascular lymphoma (IVL) is a rare subtype of extra-nodal large B-cell lymphoma involving selective growth of tumor in the vascular lumen. IVL may involve various organs including skin, gastrointestinal tract, and brain depending on the subtype. Common presentation of CNS-based IVL is dementia (60%), paralysis/paraplegia (22.1%), and seizures (13.41%). Rare presentations include aphasia(3.9%), hearing disturbances(4.3%) and psychosis(1.45%). Design/Methods: We present a case report of CNS-based IVL presenting with acute onset focal neurologic symptoms mimicking TIA and stroke. Results: A 74 year old high-functioning man with no significant past medical history presented to the emergency room with a 45 minute spell of expressive aphasia, suspicious for TIA. Ten days later he presented with recurrent symptoms and he was treated with IV tPA and transferred to a comprehensive stroke center. Over the course of the next several weeks he developed progressive hearing loss, behavioral disturbances, and further hemorrhages. MRI scan of the brain showed numerous scattered intraparenchymal microbleeds, subarachnoid and intraventricular hemorrhages. CSF analysis showed elevated protein and IgG levels. A brain biopsy was performed which revealed large B-Cell lymphoma predominantly in an intravascular distribution. Discussion: IVL is a rare form of malignancy that may mimic stroke and diagnosis is clinically difficult. CSF findings of elevated protein and oligoclonal banding have been reported as well as elevated LDH in serum. Imaging findings are often variable and include ischemic infarcts, hemorrhage, non-specific white matter lesions, meningeal enhancement, and gadolinium enhanced T1 lesions. Definitive diagnosis is made by brain biopsy. Conclusions: IVL is a rare disorder in which diagnosis is often post mortem. Since definitive diagnosis is difficult to make clinically, CNS biopsy is required. The rapid progression and association with both ischemic and hemorrhagic events may be mistaken for CNS vasculitis. This case underscores the importance of brain biopsy in rapidly progressive CNS vascular conditions. Disclosure: Dr. Elangovan has nothing to disclose. Dr. Lowe has nothing to disclose. Dr. Tiwana has nothing to disclose. Dr. Niazi has nothing to disclose. Dr. Specht has nothing to disclose. Dr. Ibrahimi has nothing to disclose. Dr. Ermak has nothing to disclose.