[1734] Cerebrovascular A-beta Amyloid in Surgical Specimens: Clinicopathological Correlates

Caterina Giannini, Jonathan M Morris, Gene G Hunder, Joseph E Parisi, Teresa J Christianson, Robert D Brown, Carlo Salvarani. Mayo Clinic, Rochester, MN; Arcispedale S. Maria Nuova, Reggio Emilia, Italy

Background: The spectrum of CNS vascular A-beta amyloid includes cerebral amyloid angiopathy (CAA), with media/adventitia A-beta deposits in small/medium size cortical and leptomeningeal arteries; A-beta-related angiitis (ABRA), with angiodestructive often granulomatous inflammation (Brain 2005;128:500-15); and CAA-related inflammation (CAA-RI), in which inflammation with giant cell reaction surrounds amyloid-laden vessels without angiodestructive features (Ann Neurol 2004;55:250-6).
Design: CNS biopsies from 62 patients with confirmed A-beta amyloid angiopathy (Surgical pathology archive 1987-2011) were re-reviewed for the presence, location and extent of vascular: A-beta deposits (leptomeningeal/cortical;intimal/medial/adventitial; segmental/circumferential); inflammation (lymphocytes/macrophages/giant cells; perivascular/transmural); and fibrinoid necrosis. Following strict pathological criteria they were classified as ABRA, CAA or CAA-RI. Clinical and imaging data were collected from the medical records.
Results: Clinical data at presentation for ABRA, CAA, CAA-RI and overall group are summarized in the Table

 ABRA (N=21)CAA (N=32)CAA-RI (N=9)Total (N=62)
Male11 (52%)15 (47%)5 (56%)31 (50%)
Female10 (48%)17 (53%)4 (44%)31 (50 %)
Age (Median, range)67 (42-84)72 (48-86)72 (53-79)70.5 (42-86)
Intracranial hemorrhage1 (5%)23 (72%)2 (22%)12 (19%)
Altered cognition5 (24%)6 (19%)2 (22%)13 (21%)
Seizure or Headache11 (52%)3 (9%)4 (44%)18 (30%)

Reason for/ time of biopsy was hematoma evacuation in 1 (5%) ABRA, 22 (69%) CAA and 2 (22%) CAA-RI. A mass lesion was present in 7 (33%) ABRA, 4 (12%) CAA and 2 (22%) CAA-RI; leptomeningeal enhancement in 9 (43%) ABRA, 2 (6%) CAA and 3 (33%) CAA-RI. At last follow up a poor outcome (Death or Rankin Score 4+) was noted in 29% of patients with ABRA, 75% with CAA, and 56% with CAA-RI.
Conclusions: CAA, ABRA and CAA-RI occur within a wide age range. As previously observed, CAA presents most often with intracranial hemorrhage. ABRA and CAA-RI likely represent a spectrum of one clinicopathological entity.
Category: Neuropathology

Monday, March 4, 2013 8:45 AM

Proffered Papers: Section G1, Monday Morning


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