Table 3.
Validation of the Diagnostic Criteria for Clinical Diagnosis of ALSP
| Number of Cases | Probable, n (%) | Possible, n (%) | Not Fulfilled, n (%) | ||
|---|---|---|---|---|---|
| Sensitivitya (%) | |||||
| Our case series | 24 | 20 (83) | 3 (13) | 1 (4) | 96 |
| Literature search | 59 | 30 (51) | 29 (49) | 0 (0) | 100 |
| Total | 83 | 50 (60) | 32 (39) | 1 (1) | 99 |
|
| |||||
| Specificity (%) | |||||
| Leukoencephalopathy without CSF1R mutation | 53 | 4 (8) | 27 (51) | 22 (42) | 42 |
| CADASIL with NOTCH3 mutation | 32 | 0 (0) | 4 (12) | 28 (88) | 88 |
ALSP, Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia; CADASIL, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy; CSF1R, colony stimulating factor 1 receptor
a
Sensitivity was calculated as a ratio of the number of cases who were diagnosed as probable or possible to the total number of cases.