Table 2.
Demographic and clinical characteristics of GD1 patients with and without Parkinsonism, after matching.
| Patients without Parkinsonism reported |
Patients with Parkinsonism reported |
p-value | |
|---|---|---|---|
| Patients enrolled | 649 | 68 | |
| Age at Gaucher diagnosisa (years) | n = 598 | n = 65 | 0.0208 |
| Median (25th, 75th) | 29 (14, 46) | 41 (19, 49) | |
| Mean (SD) | 31 (20) | 37 (20) | |
| Min, max | 0, 85 | 3, 80 | |
| Age at Gaucher diagnosisa, n (%) | n = 598 | n = 65 | 0.0269 |
| Prenatal to <10 years | 109 (18.2) | 10 (15.4) | |
| 10 to <20 years | 95 (15.9) | 7 (10.3) | |
| 20 to <30 years | 110 (18.4) | 6 (8.8) | |
| 30 to <40 years | 89 (14.9) | 8 (11.8) | |
| 40 to <50 years | 75 (12.5) | 18 (26.5) | |
| 50 to <60 years | 62 (10.4) | 7 (10.3) | |
| 60 to <70 years | 37 (6.2) | 7 (10.3) | |
| 70 years or more | 21 (3.5) | 2 (2.9) | |
| Treatment status, n (%) | n = 649 | n = 68 | 0.0808 |
| Ever on imiglucerase | 565 (87.1) | 54 (79.4) | |
| Never on imiglucerase | 84 (12.9) | 14 (20.6) | |
| Age at first infusion (years) | n = 564 | n = 54 | 0.0028 |
| Median (25th, 75th) | 47 (38, 58) | 54 (47, 63) | |
| Mean (SD) | 48 (14) | 54 (13) | |
| Min, max | 9, 87 | 15, 80 | |
| Deceased, n (%) | n = 649 | n = 68 | 0.0573 |
| Yes | 88 (13.6) | 15 (22.1) | |
| No | 561 (86.4) | 53 (77.9) | |
| Age at death (years) | n = 83 | n = 13 | 0.0018 |
| Median (25th, 75th) | 62 (50, 75) | 73 (71, 75) | |
| Mean (SD) | 63 (15) | 72 (8) | |
| Min, max | 20, 89 | 50, 85 | |
| Ethnicity, n (%) | n = 368 | n = 57 | 0.4591 |
| Caucasian, non-Jewish | 109 (29.6) | 15 (26.3) | |
| Jewish, Ashkenazi | 241 (65.5) | 37 (64.9) | |
| All others | 18 (4.9) | 5 (8.8) | |
| Splenectomy status, n (%) | n = 649 | n = 68 | 0.0128 |
| Never splenectomized | 356 (54.9) | 48 (70.6) | |
| Ever splenectomized | 293 (45.1) | 20 (29.4) | |
| Age at onset of Parkinsonism (years) | n = 61 | ||
| Median (25th,75th) | 56 (49, 64) | ||
| Mean (SD) | 57 (10) | ||
| Min, max | 30, 80 |
a
Patients with no diagnosis date or with diagnosis date earlier than 1 year prior to their birth were excluded from the analysis.