Table 3.
Comparison of clinical and MRI characteristics in perivenous versus confluent demyelination cohorts
| Clinical and MRI characteristics | PVD (n = 13) | CD (n = 91) | Pa |
|---|---|---|---|
| Median age, years (range) | 44 (5-68) | 39 (8–69) | 0.24 |
| Female:male | 10:3 | 47:44 | 0.14 |
| Preceding infection/vaccination, n (%) | 4 (31) | 14 (15) | 0.09 |
| Multifocal T2 MRI lesions, n (%)b | 10 (77) | 54 (63) | 0.18 |
| Multifocal enhancing MRI lesions, n (%) | 4 of 10 (40%) | 25 (27) | <0.001 |
| Polysymptomatic, n (%)b | 11 (85) | 67 (74) | 0.51 |
| Encephalopathy, n (%)b | 10 (77) | 23 (25) | <0.001 |
| Depressed level of consciousness, n (%) | 8 (62) | 1 (1)c | <0.001 |
| Bilateral optic neuritis, n (%) | 2 (15%) | 1 (1) | 0.04 |
| Headache, n (%) | 8 (62) | 8 (9) | <0.001 |
| Meningismus, n (%) | 2 (15) | 1 (1) | 0.04 |
| Seizure, n (%) | 3 (23) | 5 (6) | 0.06 |
| Cerebellar, n (%) | 3 (23) | 31 (34) | 0.54 |
| Brainstem, n (%) | 2 (15) | 25 (27) | 0.51 |
| Cognitive, n (%)d | 10 (77) | 35 (38) | 0.01 |
| Motor, n (%) | 7 (54) | 47 (52) | 1.00 |
| Sensory syndrome, n (%) | 4 (31) | 36 (40) | 0.76 |
| KIDMUS MRI criteria, n (%)e | 0 (0) | 15 (17) | 0.35 |
| Absence of any KIDMUS MRI criteria, n (%) | 7 (64) | 23 (26) | 0.01 |
| Median days symptom onset to biopsy/autopsy (range) | 27 (3 days to 1.9 years) | 45 (4 days to 27.6 years) | 0.13 |
| Cerebrospinal fluid | |||
| White blood cells/mm3, median (range) | 19.0 (1.0–540.0) | 3.0 (0.0–1250.0) | 0.04 |
| Protein, median mg/dl (range) | 40.9 (10.0–215.0) | 40.5 (15.0–175.0) | 0.92 |
| Elevated immunoglobulin G synthesis rate, n (%) | 1 (14) | 17 (40) | 0.66 |
| Oligoclonal bands, n (%) | 1 of 7 (14) | 12 of 54 (22) | 1.0 |
| Follow up | |||
| Duration, median (range) | 9.6 (1.4–16.5) | 2.9 (0.1–18.8) | 0.25 |
| EDSS at presentation, median (range) | 9 (0–9.5) | 3 (0–9) | 0.003 |
| EDSS last follow up (living patients), median (range) | 3.5 (0–7) | 2.5 (0–8) | 0.53 |
PVD = perivenous demyelination; CD = confluent demyelination; EDSS = Expanded Disability Status Scale.
a The Wilcoxon rank sum test was used to compare continuous measures and Fishers exact test for discrete measures.
b Three major International Paediatric Multiple Sclerosis Study Group ADEM criteria (IPMSSG) criteria for patients presenting with first attacks of idiopathic inflammatory demyelination.
c Confounded by benzodiazepine and urosepsis.
d Cognitive includes depressed level of consciousness.
e Sole presence of well defined lesions AND ovoid lesions perpendicular to the long axis of corpus callosum.