Table 2.
Clinical and pathological features of ET cases grouped by family history
| Variables | Sporadic ETa | Familial ET
|
|||
|---|---|---|---|---|---|
| Liberal criterionb | Conservative criterionc | Sporadic ET vs. familial ET liberal criterion | Sporadic ET vs. familial ET conservative criterion | ||
| n | 33 | 27 | 20 | ||
| Age at death (years) | 85.6 ± 7.3 | 86.6 ± 5.8 | 87.5 ± 6.1 | 0.53d | 0.33d |
| Age of tremor onset (years) | 42.7 ± 23.4 | 45.6 ± 19.5 | 45.2 ± 20.0 | 0.61d | 0.70d |
| Duration of symptoms (years) | 42.8 ± 22.0 | 41.0 ± 21.3 | 42.3 ± 21.7 | 0.75d | 0.93d |
| Gender | |||||
| Male | 12 (36.4%) | 10 (37.0%) | 9 (45.0%) | 0.96e | 0.55e |
| Female | 21 (63.6%) | 17 (63.0%) | 11 (55.0%) | ||
| Total tremor scores | 25.3 ± 7.1 | 24.5 ± 5.7 | 22.9 ± 5.8 | 0.65d | 0.27d |
| Purkinje cell counts | 9.09 ± 1.48 | 8.18 ± 2.17 | 8.24 ± 1.50 | 0.059d | 0.047d |
| Torpedo counts | 18.21 ± 18.12 | 11.93 ± 9.24 | 13.15 ± 9.86 | 0.26f | 0.53f |
| Median = 15.0 | Median = 9.00 | Median = 9.00 | |||
| Heterotopic Purkinje cell counts | 9.58 ± 11.21 | 5.30 ± 4.94 | 6.45 ± 5.24 | 0.14f | 0.62f |
| Median = 6.00 | Median = 4.00 | Median = 6.00 | |||
| Hairy basket ratings | 1.98 ± 0.80 | 2.02 ± 0.78 | 2.08 ± 0.69 | 0.84f | 0.70f |
| Median = 2.00 | Median = 2.00 | Median = 2.00 | |||
| Purkinje cell axonal recurrent collateral counts | 2.00 ± 1.69 | 1.76 ± 1.39 | 1.94 ± 1.61 | 0.77f | 0.91f |
| Median = 1.66 | Median = 1.11 | Median = 1.29 | |||
| Purkinje cell thickened axonal profile counts | 2.14 ± 2.54 | 1.37 ± 2.06 | 1.70 ± 2.42 | 0.037f | 0.17f |
| Median = 1.57 | Median = 0.78 | Median = 0.92 | |||
| Purkinje cell axonal branching counts | 0.51 ± 0.50 | 0.25 ± 0.22 | 0.25 ± 0.23 | 0.045c | 0.079c |
| Median = 0.49 | Median = 0.20 | Median = 0.16 | |||
Values represent mean ± standard deviation or number (percentage), and for variables with non-normal distribution, the median is reported as well Mean number of Purkinje cell counts (PCs) per ×100 microscopic field, among 15 sampled fields
a
Sporadic ET requires absence of either first- or second-degree relative with reported ET
b
Liberal criteria requires at least one first- or second-degree relative with reported ET
c
Conservative criteria requires at least one first-degree relative with reported ET
d
Student’s t test
e
Chi-square test
f
Independent samples Mann-Whitney U test
The italicized entries are statistically significant