Clinical Reasoning: A 55-Year-Old Woman With Recurrent Episodes of Aphasia and Vision Changes

A 55-year-old woman presented with recurrent episodes of headache, vision changes and language disturbances. Brain MRI showed multifocal white matter lesions, microhemorrhages, and enlarged perivascular spaces. After an extensive and unrevealing workup, she underwent a biopsy of brain and meninges that revealed thick and hyalinized leptomeningeal and cortical vessel walls that were strongly positive for ß-amyloid by immunohistochemical staining, suggestive of cerebral amyloid angiopathy (CAA). CAA can present as a spectrum of inflammatory responses to the deposition of amyloid-ß in the vessel walls. Her clinical presentation, radiological and histopathological findings supported a diagnosis of probable CAA-related inflammation (CAA-ri). Although an uncommon entity, it is important to recognize it because most patients respond to immunosuppressive therapy.

P.087 Successful response to rituximab in a patient with Aβ related angiitis

Background: Aβ-related angiitis (AβRA) is a rare presentation of cerebral amyloid angiopathy, where vasculitis results from an auto-immune reaction to amyloid deposits in leptomeningeal and cortical vessel walls. Anti-CD20 monoclonal antibodies, such as rituximab, have demonstrated efficacy in systemic small vessel vasculitides, particularly in refractory cases. The efficacy of rituximab in AβRA remains unknown. Methods: Patient chart, functional measures, and laboratory findings were reviewed from the time of patient admission until 12 months after discharge. Results: A 61-year-old man presented with headache and altered mental status. Brain MRI revealed multiple cortical infarcts, leptomeningeal enhancement, and cortical microbleeds, and brain biopsy ultimately confirmed the diagnosis of AβRA. The patient developed new ischemic lesions despite corticosteroid pulse, and intravenous cyclophosphamide was halted after four weeks due to iatrogenic acute hepatitis. Rituximab was initiated and led to sustained clinical improvement with no subsequent relapses. Maintenance therapy involved gradually tapered low-dose oral steroids and rituximab at 6- and 12-months post-induction. Conclusions: This report suggests that rituximab may be effective in inducing remission and preventing relapses in biopsy-proven case of AβRA. Controlled studies are needed to better assess the efficacy and tolerability of anti-CD20 antibodies in cerebral vasculitis.

B-Flow Sonography vs. Color Doppler Sonography for the Assessment of Vascularity in Pediatric Kidney Transplantation

Objective To compare B-flow sonography (BFS) with color Doppler sonography (CDS) for imaging of kidney transplant vascularization in children. Patients and Methods All children receiving a kidney transplantation who underwent a protocol-based ultrasound examination (Loqiq 9, GE Medical Systems, Milwaukee, WI, USA) using the BFS and CDS technique with equal settings and probe position between January 2013 and January 2016 were retrospectively assessed (n = 40). The obtained datasets were visually graded according to the following criteria: (I) delineation of the renal vascular tree (Grade 1 – clear demarcation of interlobar, together with arcuate and interlobular vessels; Grade 2 – clear demarcation of interlobar and cortical vessels, but no distinction of interlobular from arcuate vessels; Grade 3 – only clear demarcation of interlobar vessels, Grade 4 – insufficient demarcation) (II) delineation of cortical vessel density in ventral, lateral, and dorsal part of the transplant, (III) smallest vessel-capsule distance, and (IV) maximum cortical vessel count. Comparison between methods was performed using Fisher’s exact and paired sample t-tests. Results Applying a curved transducer (C1–6), BFS showed superior delineation of the renal vascular tree (p < 0.001), a lower vessel-capsule distance (p < 0.001), a higher cortical vessel count (p < 0.001), and a higher cortical vessel density in the superficial cortex (p = 0.01) than CDS. In the dorsal and lateral aspects of the transplant, cortical vessel density was lower with BFS (both p < 0.001). Using a linear high-resolution transducer (ML 6–15), no significant differences between the methods were found. Conclusion Improved imaging of kidney transplant vascularization can be achieved in children by adding BFS to a standard protocol. The BFS technique is especially beneficial for overall assessment of the renal vascular tree together with the extent of cortical vascularization on curved array images. Key points: Citation Format