2026 American Academy of Neurology Abstract Website

Objective:

To describe a rare case of Evans syndrome complicated by bilateral thalamic infarcts, emphasizing the paradoxical risk of thrombosis despite thrombocytopenia and the importance of early multidisciplinary management.

Background:

Evans syndrome is a rare autoimmune disorder characterized by autoimmune hemolytic anemia and thrombocytopenia, with a prevalence of 21.3 per million. Its pathophysiology is incompletely understood but may involve platelet-derived microparticles (PMPs), promoting thrombin generation and paradoxical thrombosis despite low platelet counts. Evans syndrome may be primary or secondary to infections, lymphoproliferative, or autoimmune disorders, with mortality of ~20% and thrombotic events in up to 20% of cases. Cytopenias, immune-mediated hypercoagulability, and vascular factors may contribute to atypical stroke presentations and complicate management.

Design/Methods:

A 47-year-old female presented with chest pain, dyspnea, generalized weakness, and dark urine in the context of thrombocytopenia. Labs revealed anemia (Hb 8.7 g/dL, Hct 24.7%), thrombocytopenia (65 ×10³/μL), and hyperbilirubinemia (1.5 mg/dL). Bone marrow biopsy confirmed Evans syndrome, and therapy with high-dose steroids, IVIG, and rituximab was initiated. During hospitalization, she developed an acute change in mental status. Brain MRI revealed bilateral thalamic and left centrum semiovale infarcts. MRA identified an artery of Percheron variant, and MRV showed a filling defect in the left transverse sinus without flow limitation.

Results:

Due to the patient’s profound thrombocytopenia, aspirin was contraindicated for secondary stroke prevention. Given the likely hematologic etiology of the infarcts, statin therapy was not indicated. The patient was subsequently started on plasmapheresis.

Conclusions:

This case highlights bilateral thalamic infarcts in Evans syndrome, demonstrating stroke risk even with severe thrombocytopenia. Early multidisciplinary evaluation is essential. IVIG therapy may carry a rare risk of thromboembolism related to transient hyperviscosity and prothrombotic changes. To our knowledge, this is the first reported case of bilateral thalamic infarcts as a complication of Evans syndrome.