PS-R06-6: CEREBRAL AMYLOID ANGIOPATHY-RELATED INFLAMMATION PRESENTING WITH POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME

Abstract

Background: Cerebral amyloid angiopathy related inflammation (CAAri) is a rare CAA variant, characterized by headaches, seizures, acute or subacute encephalopathy, or focal neurological deficits. Radiologically, CAAri presents with widespread white matter lesions on brain MRI in addition to the hemorrhagic imaging features of CAA. We report a patient with CAAri who showed posterior reversible encephalopathy syndrome. Case Report: A 77 year old man presented with generalized tonic clonic seizure that persisted for 30 minutes and decreased in consciousness level. He had past medical history of dementia, diabetes and hypertension. His initial blood pressure was 170/90 mmHg and pulse rates were 144/minute. On neurological examination, his consciousness level was stupor and there are no lateralizing signs. Cerebrospinal fluid analysis was unremarkable except elevated protein level of 75 mg/dL. Diffusion weighted images (DWI) and fluid attenuated inversion recovery (FLAIR) images showed diffuse hyperintensities involving the bilateral temporoparietooccipital lobes. Gradient echo images revealed multiple microbleeds in bilateral cortical and subcortical regions as can be seen in CAA. A diagnosis of CAAri was considered, and the patient was started on steroids and valproic acid with clinical improvement. Nine days after admission, follow up DWI and FLAIR images demonstrated improvement of previously shown lesions. Conclusions: Aside from the most common subacute presentations, CAAri can have a PRES like presentation. It is hypothesised that in this form, CAAri causes altered vascular autoregulation and impaired blood brain barrier integrity in the face of precipitating factors such as hypertension.