Table 2.
Incidence of dementia-related diagnoses and drug purchases among persons with neurofibromatosis type 1 (NF1) and matched controls during 1998–2014.
| Individuals with NF1 | Controls | ||||
|---|---|---|---|---|---|
| Events, n (males/females) | Age at first dementia-related encounter, years, mean (SD) | Events, n (males/females) | Age at first dementia-related encounter, years, mean (SD) | HR (95% CI), P | |
| Alzheimer disease, any type of dementia or antidementia drug (ICD-10 G30, F00–F03, ATC N06D) | 16 (8/8) | 74.2 (9.0) | 165 (63/102) | 77.6 (7.1) | 1.67 (1.00 to 2.80), 0.050 |
| Alzheimer disease (ICD-10 G30) | 10 (6/4) | 75.6 (10.0) | 61 (19/42) | 77.3 (7.6) | 2.88 (1.47 to 5.66), 0.002 |
| Any type of dementia (ICD-10 F00–F03) | 8 (4/4) | 76.9 (6.8) | 60 (26/34) | 77.3 (8.6) | 2.42 (1.15 to 5.09), 0.020 |
| Dementia in Alzheimer disease (ICD-10 F00) | 6a | 75.7 (7.8) | 26 (7/19) | 77.5 (8.6) | 4.04 (1.65 to 9.93), 0.002 |
| Vascular dementia (ICD-10 F01) | <3a | - | 14 (9/5) | 76.6 (7.8) | - |
| Dementia in other diseases classified elsewhere (ICD-10 F02) | <3a | - | 5a | 72.1 (3.0) | - |
| Unspecified dementia (ICD-10 F03) | 3a | 82.2 (1.7) | 13 (3/10) | 80.0 (8.9) | 5.04 (1.39 to 18.28), 0.014 |
| Antidementia drug (ATC N06D) | 15 (7/8) | 74.9 (9.6) | 129 (48/81) | 78.2 (6.1) | 2.01 (1.18 to 3.44), 0.011 |
| Anticholinesterase (ATC N06DA) | 11 (6/5) | 75.7 (10.8) | 115 (42/73) | 78.1 (5.9) | 1.62 (0.87 to 3.01), 0.129 |
| Other antidementia drug (ATC N06DX) | 8a | 77.4 (4.8) | 59 (19/40) | 79.1 (6.3) | 2.50 (1.18 to 5.28), 0.017 |
All subcodes of the ICD-10 and ATC codes were included. An event is defined as the second encounter of interest (hospital visit, drug purchase or either one, depending on analysis) to avoid any coding errors. The mean ages are shown for the first encounter of interest during the follow-up.
ATC Anatomical Therapeutic Chemical classification, CI confidence interval, HR hazard ratio, ICD-10 International Standard Classification of Diseases—10th revision, NF1 neurofibromatosis type 1, SD standard deviation.
aSex-stratified and total numbers <3 are not shown to protect patient privacy.