Summary of clinical features and key investigation findings in reported cases of iatrogenic CAA
| Previously reported cases (n=20) | Cases in this report (n=3) | All (n=23) | |||
| Age at first presentation, mean (SD), years | 36.9 (8.3) | 43.0 (3.6) | 37.7 (8.1) | ||
| Sex, male, n (%) | 15 (75.0) | 2 (66.7) | 17 (73.9) | ||
| Age at first exposure, mean (range), years | 3.3 (0.1 to 20.0) | 4.0 (3.0 to 5.0) | 3.3 (0.1 to 20.0) | ||
| Latency, mean (range), years | 33.5 (25.0 to 46.0) | 39.0 (36.0 to 42.0) | 34.3 (25.0 to 46.0) | ||
| Exposure, n (%) | Dura mater | 8 (40.0) | 2 (66.7) | 10 (43.5) | |
| Cadaveric dura mater | 7 (35.0) | 2 (66.7) | 9 (39.1) | ||
| Neurosurgery | 17 (85.0) | 1 (33.3) | 18 (78.3) | ||
| ApoE genotype*, n (%) | ε3 | 18 (100.0) | 3 (100.0) | 21 (100.0) | |
| ε3/ε3 | 12 (66.7) | 2 (66.7) | 14 (66.7) | ||
| Presenting symptom, n (%) | ICH (including cSAH) | 17 (85.0) | 2 (66.7) | 19 (82.6) | |
| Seizures | 4 (20.0) | 0 (0.0) | 4 (17.4) | ||
| Associated symptoms, n (%) | ICH | 17 (85.0) | 3 (100.0) | 20 (87.0) | |
| Recurrent ICH | 14 (70.0) | 1 (33.3) | 15 (65.2) | ||
| Cognitive impairment | 6 (30.0) | 3 (100.0) | 9 (39.1) | ||
| Seizures | 6 (30.0) | 0 (0.0) | 6 (26.1) | ||
| Investigations | |||||
| MRI features of CAA | cSS | Reported, n | 10 | 2 | 12 |
| Present, n (%) | 8 (80.0) | 1 (50.0) | 9 (75.0) | ||
| CMB | Reported, n | 16 | 2 | 18 | |
| Present, n (%) | 16 (100.0) | 2 (100.0) | 18 (100.0) | ||
| Amyloid PET | Test performed, n | 9 | 1 | 10 | |
| Positive, n (%) | 9 (100.0) | 1 (100.0) | 10 (100.0) | ||
| CSF findings | Aβ−40 | Test performed, n | 1 | 0 | 1 |
| Reduced, n (%) | 1 (100.0) | 0 (0.0) | 1 (100.0) | ||
| Aβ−42 | Test performed, n | 7 | 1 | 8 | |
| Reduced, n (%) | 7 (100.0) | 1 (100.0) | 8 (100.0) | ||
| Total tau | Test performed, n | 5 | 1 | 6 | |
| High n (%) | 1 (20.0) | 1 (100.0) | 2 (33.3) | ||
| p-tau | Test performed, n | 4 | 0 | 4 | |
| High, n (%) | 0 (0.0) | 0 (0.0) | 0 (0.0) | ||
| Brain biopsy | Test performed, n | 12 | 3 | 15 | |
| CAA observed, n (%) | 12 (100.0) | 3 (100.0) | 15 (100.0) | ||
Please see online supplemental table 1 for individual details for the cases described in this report, and online supplemental table 2 for further details of published cases to date.
*ApoE genotype reported for 18 of 20 previously published cases.
ApoE, apolipoprotein E; Aβ, amyloid-beta; CAA, cerebral amyloid angiopathy; CMB, cerebral microbleed (lobar); cSAH, convexity subarachnoid haemorrhage; cSS, cortical superficial siderosis; ICH, intracerebral haemorrhage; p-tau, phospho-tau.