Amyloid Beta-Related Angiitis Presenting as Multiple Cerebral Infarcts

Abstract

Background

Amyloid beta-related angiitis (ABRA) is a rare sporadic granulomatous angiitis that includes features from both primary angiitis of the central nervous system angiitis (PACNS) and cerebral amyloid angiopathy (CAA). (Rigby et al., 2011) PACNS is clinically defined as a history of unexplained neurological symptoms in the setting of central nervous system vasculitis. (Rigby et al., 2011) CAA is pathologically defined by amyloid-beta (Aβ) deposition in the walls of arteries and arterioles. (Rigby et al., 2011) ABRA incorporates the diagnostic criteria of PACNS and distinct pathological features of CAA with the addition of intramural multinucleated giant cell infiltration and vessel wall destruction. (Rigby et al., 2011)

Case Presentation

This 67-year-old right-handed African American female with a past medical history of hypertension, diabetes, and alpha thalassemia trait presented to an outside hospital in an acute confusional state.

Case Report

The patient was transferred to our tertiary medical center after initial head CT was concerning for intraparenchymal and subarachnoid hemorrhages. Subsequent MRI brain confirmed a right thalamic hemorrhage and right cavernous hemangioma in addition to a right frontal subcortical ischemic infarct. A four-vessel diagnostic cerebral angiogram revealed severe distal stenoses in noncontiguous segments of the anterior and posterior intracranial arteries. MRI brain was repeated after the patient experienced new right sided weakness, which showed new scattered multi-territory ischemic infarcts. Laboratory studies were significant for and consistent with non-specific inflammation, and a right frontal brain biopsy was consistent with cerebral amyloid angiopathy. The patient was suspected to have ABRA, and her clinical condition stabilized yet remained disabled with corticosteroids and cyclophosphamide.

Conclusion

ABRA, an inflammatory disease related to PACNS, is a sequela of Aβ deposition in the cerebral vasculature. In this patient, we suspect that Aβ deposits in the cerebral vasculature triggered an intramural inflammatory cascade resulting in scattered ischemic infarcts. This case study highlights the unique clinical presentation, required diagnostic evaluation, and treatment options for this rare form of cerebral angiitis.