Table 2. Pathologic features comparing between PSP with and without cerebellar ataxia.
Ataxia (43) | No ataxia (61) | P value | |
---|---|---|---|
Demographic | |||
Gender, % male | 23/43 (52%) | 40/61 (65%) | 0.255 a |
Age at death | 74.8 ± 9.0 | 73.9 ± 7.4 | 0.585 b |
Pathologic features | |||
Brain weight (g) | 1120 (1060, 1195) | 1200 (1110, 1320) | 0.011 c |
Braak NFT stage | III (II, III) | II (I, III) | 0.069 c |
Thal amyloid phase | 1.0 (0, 3) | 0 (0, 3) | 0.200 c |
Ratio of Purkinje tau positive | 27/42 (64%) | 36/60 (60%) | 0.817 a |
Number of pretangles in Purkinje cell layer | 1.0 (0, 2) | 1.0 (0, 3) | 0.910 c |
Pretangles linear density (cells/mm) | 3.6 (3, 4) | 3.7 (3, 4) | 0.421 c |
Semi-quantitative analysis | |||
Coiled bodies in cerebellar white matter | 2.0 (1, 3) | 2.0 (1, 3) | 0.860 c |
Tau threads in cerebellar white matter | 2.0 (1, 3) | 1.0 (1, 2) | 0.496 c |
Coiled bodies in dentate nucleus | 1.0 (0, 1) | 1.0 (0, 1) | 0.732 c |
Tau threads in dentate nucleus | 2.0 (1, 2) | 2.0 (1, 2) | 0.332 c |
Pretangles/NFT in dentate nucleus | 2.0 (2, 3) | 3.0 (2, 3) | 0.335 c |
Pretangles/NFT in pontine base | 3.0 (2, 3) | 3.0 (2, 3) | 0.477 c |
Pretangles/NFT in inferior olivary nucleus | 2.0 (1, 2) | 2.0 (1, 2) | 0.192 c |
Genetic analysis | |||
MAPT: H1H1 genotype | 36/38 (95%) | 42/49 (86%) | 0.170 d |
MAPT: H1H2 genotype | 2/38 (5%) | 7/49 (14%) | 0.170 d |
APOE: ε4 genotype | 10/39 (26%) | 12/53 (23%) | 0.739 a |
Data are displayed as mean ± SD or median (25th, 75th range) as appropriate. Abbreviations: PSP, progressive supranuclear palsy; PSP-C, PSP, progressive supranuclear palsy with predominant cerebellar ataxia; NFT, neurofibrillary tangles;
Chi-square test,
t-test,
Mann-Whitney rank sum test,
Fisher exact test