In:
Stroke, Ovid Technologies (Wolters Kluwer Health), Vol. 50, No. Suppl_1 ( 2019-02)
Abstract:
Introduction: Cerebral amyloid angiopathy-related inflammation (CAAri) is characterized by altered cognition, headaches, and seizures and is associated with vasogenic edema and an anti-amyloid immune response on imaging and histology. Optimal treatments are poorly understood. Our objective was to analyze clinical features and treatment strategies in CAAri to identify predictors of outcome. Methods: We retrospectively reviewed the records of 56 consecutive patients with pathologically or radiographically diagnosed CAAri at Massachusetts General Hospital. Clinical, laboratory, pathology, and treatment data were collected for analysis. Inflammatory events were defined by previously described clinical and radiographic parameters. Chi-Square analyses were used to determine if treatment with immunotherapy was associated with outcomes. Results: Of the 56 subjects, 8 had vessel wall inflammation on pathology, 21 had perivascular inflammation on pathology, and 48 had probable CAAri by validated imaging criteria. Thirty-five were female, 49 presented with an inflammatory event, and the mean age at first event was 69.3+/-10.2 years. First events included headache (n=21), cognitive/behavioral change (29), focal deficit (9), seizure (20), and other (6). CSF was examined in 26 subjects after first event (6 with WBC 〉 5, 24 with protein 〉 45, 1 without abnormality). Treatments included steroids (33), cyclophosphamide (6), mycophenolate (2), and no treatment (14). After first event, clinical improvement (n=39) was more likely if treated with any immunotherapy than not (32/34 vs 7/14, p=0.001). Radiographic improvement (28) was more likely if treated than not (24/28 vs 4/14, p 〈 0.0001). Radiographic worsening (13) was less likely if treated than if not treated (3/28 vs. 10/14, p 〈 0.0001). Only 3 had clinical worsening and 0 were unchanged. At least 1 recurrent event (19) was more likely if not treated than if treated (10/13 vs 9/31, p=0.005); 10 had multiple recurrences and 1 died during an event. Conclusion: This study of the largest CAAri patient population to-date suggests treatment with immunosuppression is associated with improved clinical and radiographic outcomes and decreased recurrence of events.
Type of Medium:
Online Resource
ISSN:
0039-2499
,
1524-4628
DOI:
10.1161/str.50.suppl_1.WP426
Language:
English
Publisher:
Ovid Technologies (Wolters Kluwer Health)
Publication Date:
2019
detail.hit.zdb_id:
80381-9
detail.hit.zdb_id:
1467823-8
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