Table 1.
Demographic characteristics, clinical manifestation, and imaging features of HCBs patients.
| Metabolism (n = 1) | Inflammation (n = 11) | Degeneration (n = 42) | Neoplasm (n = 5) | Infection (n = 7) | Hereditary (n = 10) | Toxication (n = 1) | Stroke (n = 2) | χ2 | Pa | |
|---|---|---|---|---|---|---|---|---|---|---|
| Sex | ||||||||||
| Women | 0 | 6/11 (54.5%) | 33/42 (78.6%) | 3/5 (60.0%) | 3/7 (42.9%) | 2/10 (20.0%) | 1 | 1 | 2.59 | 0.134‡ |
| Men | 1 | 5/11 (45.5%) | 9/42 (21.4%) | 2/5 (40.0%) | 4/7 (57.1%) | 8/10 (80.0%) | 0 | 1 | ||
| Age (y) | 25 | 49 (40–55) | 60 (52–64) | 48 (40–68) | 30 (13–37) | 48 (37–71) | 24 | 66 (61–66) | 0.004§ | |
| Duration of disease (m) | 510 | 1 (0.47–5) | 36 (12–48) | 13.8 (3.6–13.8) | 2.0 (2.0–6.8) | 66 (21–180) | 240 | 8 (4–8) | <0.001§ | |
| Clinical manifestation (n) | ||||||||||
| Limb weakness | 0/1 (0.0%) | 7/11 (63.6%) | 0/41 (0.0%) | 0/5 (0.0%) | 0/6 (0.0%) | 0/10 (0.0%) | 1/1 (100%) | 1/2 (50.0%) | 30.15 | <0.001‡ |
| Limb numbness | 0/1 (0.0%) | 1/11 (9.1%) | 0/41 (0.0%) | 0/5 (0.0%) | 0/6 (0.0%) | 1/10 (10.0%) | 0/1 (0.0%) | 1/2 (50.0%) | 3.8 | 0.212‡ |
| Vision blurred | 0/1 (0.0%) | 4/11 (36.4%) | 0/41 (0.0%) | 0/5 (0.0%) | 0/6 (0.0%) | 0/10 (0.0%) | 0/1 (0.0%) | 0/2 (0.0%) | 16.15 | 0.002‡ |
| Parkinsonism | 0/1 (0.0%) | 0/11 (0.0%) | 21/41 (51.2%) | 0/5 (0.0%) | 0/6 (0.0%) | 3/10 (30.0%) | 0/1 (0.0%) | 0/2 (0.0%) | 9.45 | 0.002‡ |
| Cerebellar ataxia | 1/1 (0.0%) | 1/11 (9.1%) | 30/41 (73.1%) | 2/5 (40.0%) | 4/6 (66.7%) | 10/10 (100%) | 1/1 (100%) | 0/2 (0.0%) | 14.79 | <0.001‡ |
| MRI feature | ||||||||||
| T1 | 0/1 (0.0%) | 4/11 (36.4%) | 1/23 (4.3%) | 0/1 (0.0%) | 2/4 (50.0%) | 2/2 (100%) | 0/1 (0.0%) | 0/2 (0.0%) | 6.08 | 0.029‡ |
| T2 | 1/1 (100.0%) | 11/11 (100%) | 36/36 (100%) | 5/5 (100%) | 6/6 (100%) | 10/10 (100%) | 1/1 (100.0%) | 2/2 (100.0%) | / | / |
| T2 Flair | 0/1 (0.0%) | 3/11 (27.3%) | 4/36 (11.1%) | 0/1 (0.0%) | 4/6 (66.7%) | 2/3 (66.7%) | 0/1 (0.0%) | 0/2 (0.0%) | 3.42 | 0.341‡ |
| Grade | ||||||||||
| 1 | 0/1 (0.0%) | 5/11 (45.5%) | 7/36 (19.4%) | 0/5 (0.0%) | 0/6 (0.0%) | 1/10 (10.0%) | 0/1 (0.0%) | 0/2 (0.0%) | 3 | 0.118‡ |
| 2 | 0/1 (0.0%) | 0/11 (0.0%) | 5/36 (13.9%) | 0/5 (0.0%) | 0/6 (0.0%) | 3/10 (30.0%) | 0/1 (0.0%) | 0/2 (0.0%) | 1.71 | 0.322‡ |
| 3 | 1/1 (0.0%) | 4/11 (36.4%) | 13/36 (36.1%) | 2/5 (40%) | 4/6 (66.7%) | 6/10 (60.0%) | 1/1 (0.0%) | 2/2 (100%) | 0 | 1.000‡ |
| 4 | 0/1 (0.0%) | 2/11 (18.2%) | 8/36 (22.2%) | 3/5 (60%) | 2/6 (33.3%) | 0/10 (0.0%) | 0/1 (0.0%) | 0/2 (0.0%) | 0.08 | 1.000‡ |
| 5 | 0/1 (0.0%) | 0/11 (0.0%) | 3/36 (8.3%) | 0/5 (0.0%) | 0/6 (0.0%) | 0/10 (0.0%) | 0/1 (0.0%) | 0/2 (0.0%) | 0.98 | 1.000‡ |
| MCP-H | 0/1 (0.0%) | 2/11 (18.2%) | 4/36 (11.1%) | 3/5 (60%) | 4/6 (66.7%) | 5/10 (50.0%) | 0/1 (0.0%) | 0/2 (0.0%) | 0.38 | 0.614‡ |
| P-A | 1/1 (100.0%) | 0/11 (0.0%) | 31/36 (86.1%) | 4/5 (80%) | 1/7 (14.3%) | 8/10 (80.0%) | 0/1 (0.0%) | 0/2 (0.0%) | 27.83 | <0.001‡ |
| MCP-A | 0/1 (0.0%) | 0/11 (0.0%) | 35/36 (97.2%) | 4/5 (80%) | 1/7 (14.3%) | 9/10 (90.0%) | 1/1 (100.0%) | 0/2 (0.0%) | 41.89 | <0.001‡ |
| Cerebellar atrophy | 0/1 (0.0%) | 0/11 (0.0%) | 36/36 (100%) | 4/5 (80%) | 2/7 (28.6%) | 9/10 (90.0%) | 1/1 (100.0%) | 0/2 (0.0%) | 47 | <0.001‡ |
| Treatment& response | ||||||||||
| Etiological Treatment | Chenodeoxycholic acid | Methylprednisolone | N/A | N/A | Immunosuppressive | N/A | N/A | Antiplatelet | ||
| Response | N/A | 3/3 (100.0%) | N/A | N/A | 1/1 (100.0%) | N/A | N/A | N/A | ||
a
Comparison was done between inflammation and degeneration groups. Data are n (%), median (IQR).
†Based on χ2 test with significance level of 0.05 (two-tailed). Based on Fisher's exact test (if any cell has fewer than 10 observations) with level of significance set to 0·05 (two-tailed).
§
Based on Mann–Whitney test with significance level of 0.05.
MCP-H, middle cerebellar peduncle hyperintensity; IQR, interquartile range; P-A, pons atrophy; MCP-A, middle cerebellar peduncle atrophy; m, month; y, year.