To demonstrate the unique presentation of biopsy-proven A Beta Related Angiitis (ABRA).

Initial CT head was significant for bilateral subarachnoid hemorrhage and brain MRI showed innumerable non-enhancing diffusion restricting lesions consistent with multiple cortical and sub-cortical strokes, without clear susceptibility artifact. Initial serum workup including rheumatological panel was unremarkable aside of mild leukocytosis (12k). Lumbar puncture showed 69 WBCs, 2 RBCs and protein of 1140 mg/dL. Sedimentation rate was 17 and C Reactive Protein was 47. Spinal fluid cultures and serologies were all negative for infection. Continuous EEG showed diffuse slowing without epileptiform activity. Empirical coverage with broad spectrum antibiotics, antivirals and antifungals failed to provide any benefit. Two independent conventional angiograms failed to demonstrate any vessel abnormalities concerning for vasculitis. Given the high suspicion of primary CNS vasculitis involving small vessels patient ultimately underwent a brain biopsy, confirming ABRA. Patient responded to combine pulse steroids intravenous immunoglobulins and was able to be extubated. Unfortunately, patient suffered a subsequent abrupt neurological decline secondary to a new large intraparenchymal and intraventricular hemorrhage proceeding to death.

Vasculitis should be considered in cases with progressive central neurological symptoms and diffuse lesions (ischemic or hemorrhagic) with no clear etiology identified on extensive work up. Biopsy proves useful in such cases when angiography and laboratory results are also negative.