Pattern characteristics and analysisa
| Subpatterns | P Value | Pattern 1 Overall | Pattern 2 | P Value | |||
|---|---|---|---|---|---|---|---|
| 1.1 | 1.2 | 1.3 | |||||
| Median age at onset (mo) | 45.4 | 66 | 80.5 | .5 | 55.5 | 16 | .004 |
| Symptoms | |||||||
| Seizures | 11/21 (52%) | 4/5 (80%) | 4/6 (67%) | .5 | 19/32 (59%) | 9/25 (36%) | .08 |
| Encephalopathy | 11/21 (52%) | 1/5 (20%) | 4/6 (67%) | .3 | 16/32 (50%) | 6/25 (24%) | .04 |
| Gait ataxia | 5/21 (24%) | 3/5 (60%) | 3/6 (50%) | .08 | 12/32 (34%) | 0 | .001 |
| Limb weakness | 6/21 (29%) | 1/5 (20%) | 1/6 (17%) | .8 | 8/32 (25%) | 1/25 (4%) | .03 |
| Dysarthria | 0 | 2/5 (40%) | 1/6 (17%) | .01 | 3/32 (9%) | 1/25 (4%) | .4 |
| Diplopia | 01/21 (5%) | 2/5 (40%) | 2/6 (33%) | .06 | 5/32 (16%) | 0 | .03 |
| Abnormal CSF | 18/20 (90%) | 4/5 (80%) | 5/5 (100%) | .6 | 27/30 (90%) | 15/20(75%) | .2 |
| Proteinosis | 13 (65%) | 4 (80%) | 4 (80%) | .5 | 21 (70%) | 11 (55%) | .3 |
| Pleocytosis | 10 (50%) | 4 (80%) | 5 (100%) | .08 | 19 (63%) | 6 (30%) | .04 |
| Pooled mutation group | 4/6 (67%) | 2/3 (67%) | 3/3 (100%) | .32 | 9/12 (75%) | 13/13 (100%) | .09 |
| Mortality | 9/20 (45%) | 3/5 (60%) | 2/6 (33%) | .3 | 14/31 (45%) | 5/24 (21%) | .06 |
| Deficit-free at follow-up | 8/20 (40%) | 1/5 (20%) | 1/6 (17%) | .1 | 10/31 (32%) | 13/25(52%) | .06 |
↵a Data collected are both continuous and categorical, and the analysis method used has been referred to under statistical analysis heading.