Table 1.
Clinico-radiological characteristics of the subgroups of patients with TDL.
| Parameter analyzed | A: Marburg-like TDL n = 4 |
B: Monophasic TDL n = 7 |
C: Recurrent TDL n = 12 |
D: MS →TDL n = 16 |
E: TDL →MS n = 5 |
Statistical differences |
|---|---|---|---|---|---|---|
| Clinical and biological characteristics of the subgroups | ||||||
| Demographics | ||||||
| Age at disease initiation, years, mean (± SD) | 41 (12.36) | 34.14 (13.23) | 33.42 (11.41) | 34.06 (7.629) | 39 (15.60) | NS |
| Age at TDL presentation, years, mean (± SD) | 41 (12.36) | 37.71 (9.759) | 34.92 (11.91) | 40.63 (9.186) | 39 (15.60) | NS |
| Sex, female (%) | 4/4 (100) | 5/7 (71) | 7/12 (58) | 11/16 (69) | 3/5 (40) | NS |
| Positive family history of demyelinating disease (%) | 0/4 (0) | 1/7 (14) | 0/12 (0) | 4/16 (25) | 0/5 (0) | NS |
| General clinical data | ||||||
| Follow-up from disease onset, months, mean (± SD) | 57 (56.70) | 68.57 (33.99) | 54.25 (43.03) | 104.9 (106.6) | 29.6 (18.96) | B versus D; p = 0.0278, E versus D; p = 0.0377 |
| Number of clinical attacks during follow-up, mean (± SD) | 1.25 (0.5000) | 1 (0.000) | 3.25 (1.485) | 2.563 (0.8921) | 2 (0.000) | A versus C; p = 0.0192, A versus D; p = 0.0043, A versus E; p = 0.0476, B versus C; p = 0.0003, B versus D; p < 0.0001, B versus E; p = 0.0013 |
| MS criteria fulfilled during follow-up (%) | 2/4 (50) | 2/7 (29) | 12/12 (100) | 16/16 (100) | 5/5 (100) | NS |
| EDSS score at first tumefactive attack, mean (± SD) | 3.5 (1.732) | 3.357 (1.864) | 3.792 (1.658) | 3.719 (1.366) | 2.4 (0.9618) | NS |
| CSF studies | ||||||
| WBC, cells/μL, mean (± SD) | 0 (0.000) | 12.6 (9.555) | 1.286 (1.254) | 7.8 (7.510) | 11.5 (16.26) | B versus C; p = 0.0189, C versus D; p = 0.0169 |
| Protein, mg/dL, mean (± SD) | 35 (0.000) | 50.6 (10.88) | 41.29 (11.40) | 45.9 (29.77) | 26 (14.14) | NS |
| Glucose, mg/dL, mean (± SD) | 70 (0.000) | 62.2 (9.654) | 61.14 (8.915) | 62.2 (10.65) | 65.5 (0.7071) | NS |
| IgG index, mean (± SD) | 0.4 (0.000) | 0.996 (0.3743) | 0.5229 (0.09517) | 0.871 (0.4021) | 0.855 (0.4596) | B versus C; p = 0.0101, C versus D; p = 0.0452 |
| Oligoclonal bands, (%) | 0/1 (0) | 5/6 (83) | 4/10 (40) | 11/12 (92) | 1/3 (33) | C versus D; p = 0.0201 |
| MRI characteristics of the subgroups | ||||||
| A. Number of TDLs at disease onset with TDL | ||||||
| Solitary (%) | 4/4 (100) | 5/7 (71) | 9/12 (75) | 13/16 (81) | 5/5 (100) | NS |
| Number of TDLs, mean (± SD) | 1.000 (0.000) | 1.286 (0.4880) | 1.333 (0.6513) | 1.25 (0.5774) | 1 (0.000) | NS |
| B. Radiological characteristics at disease onset with TDL | ||||||
| Size of all TDLs, cm, mean (± SD) | 7.488 (2.196) | 3.771 (1.363) | 3.338 (0.7699) | 2.813 (0.5968) | 2.482 (0.4187) | A versus B; p = 0.0212, A versus C; p = 0.0011, A versus D; p = 0.0004, A versus E; p = 0.0159, C versus E; p = 0.0357 |
| Presence of mass effect (%) | 3/4 (75) | 0/7 (0) | 1/12 (8) | 1/16 (6) | 0/5 (0) | A versus B; p = 0.0242, A versus C; p = 0.0269, A versus D; p = 0.0134, A versus E; p = 0.0476 |
| Presence of edema (%) | 3/4 (75) | 2/7 (29) | 3/12 (25) | 5/16 (31) | 0/5 (0) | A versus E; p = 0.0476 |
| Presence of T2w hyperintensity (%) | 4/4 (100) | 3/7 (43) | 4/12 (33) | 11/16 (69) | 1/5 (20) | A versus E; p = 0.0476 |
| Barkhof criteria (%) | 0/4 (0) | 2/7 (29) | 6/12 (50) | 16/16 (100) | 3/5 (60) | A versus D; p = 0.0035, B versus D; p = 0.0006, C versus D; p = 0.0025, D versus E; p = 0.0476 |
| Spine MRI involvement (%) | 1/4 (25) | 2/7 (29) | 6/12 (50) | 10/16 (63) | 2/5 (40) | NS |
| Presence of LETM (%) | 0/4 (0) | 0/7 (0) | 2/12 (17) | 2/16 (13) | 0/5 (0) | NS |
| PVWM involvement (%) | 2/4 (50) | 4/7 (57) | 8/12 (67) | 16/16 (100) | 3/5 (60) | A versus D; p = 0.0316, B versus D; p = 0.0198, C versus D; p = 0.0242, D versus E; p = 0.0476 |
| Atrophy (%) | 0/4 (0) | 1/7 (14) | 3/12 (25) | 10/16 (63) | 2/5 (40) | NS |
| C. Enhancement pattern of disease onset with TDL | ||||||
| Presence of GD+ in TDL (%) | 4/4 (100) | 4/7 (57) | 10/12 (83) | 15/16 (94) | 4/5 (80) | NS |
| Homogeneous GD+ (%) | 1/4 (25) | 1/7 (14) | 2/12 (17) | 1/16 (6) | 0/5 (0) | NS |
| Heterogeneous GD+ (%) | 3/4 (75) | 2/7 (29) | 0/12 (0) | 5/16 (31) | 1/5 (20) | A versus C; p = 0.0071 |
| Closed ring, percentage (%) | 0/4 (0) | 0/7 (0) | 4/12 (33) | 6/16 (38) | 0/5 (0) | NS |
| Open ring (%) | 0/4 (0) | 0/7 (0) | 4/12 (33) | 1/16 (6) | 3/5 (60) | B versus E; p = 0.0455, D versus E; p = 0.0276 |
| Nodular (%) | 1/4 (25) | 1/7 (14) | 1/12 (8) | 1/16 (6) | 0/5 (0) | NS |
| Punctuate (%) | 0/4 (0) | 0/7 (0) | 1/12 (8) | 2/16 (13) | 0/5 (0) | NS |
| D. Radiological characteristics after acute treatment | ||||||
| Size of all TDLs, cm, mean (± SD) | 2.125 (3.005) | 3.147 (0.8418) | 2.052 (1.049) | 1.773 (0.9438) | 1.75 (0.3820) | B versus D; p = 0.0315 |
| Enhancement (%) | 0/2 (0) | 1/3 (33) | 1/9 (11) | 1/10 (10) | 0/3 (0) | |
| E. Radiological characteristics at last follow-up | ||||||
| Size of all TDLs, cm, mean (± SD) | 6.565 | NA | NA | 1.082 (0.8824) | 1.195 (0.3182) | NS |
| Enhancement (%) | 1/2 (50) | NA | NA | 0/6 (0) | 0/2 (0) | NS |
| Treatment strategies in the subgroups | ||||||
| First line treatment after TDL | ||||||
| Corticosteroids (%) | 4/4 (100) | 7/7 (100) | 12/12 (100) | 16/16 (100) | 5/5 (100) | NS |
| Additional plasma exchange (%) | 0/4 (0) | 2/7 (29) | 3/12 (25) | 1/16 (6) | 0/5 (0) | NS |
| Second line treatment | ||||||
| A. Immunomodulatory therapeutic approach | ||||||
| Glatiramer acetate (%) | 2/4 (50) | 1/7 (14) | 5/12 (42) | 2/16 (13) | 1/5 (20) | NS |
| Teriflunomide (%) | 0/4 (0) | 0/7 (0) | 0/12 (0) | 2/16 (13) | 0/5 (0) | NS |
| Fingolimod, percentage (%) | 0/4 (0) | 0/7 (0) | 0/12 (0) | 1/16 (6) | 1/5 (20) | NS |
| Natalizumab (%) | 0/4 (0) | 0/7 (0) | 1/12 (8) | 1/16 (6) | 1/5 (20) | NS |
| Alemtuzumab (%) | 0/4 (0) | 0/7 (0) | 1/12 (8) | 2/16 (13) | 1/5 (20) | NS |
| B. Immunosuppressive therapeutic approach | ||||||
| Cyclophosphamide (%) | 4/4 (100) | 3/7 (43) | 6/12 (50) | 3/16 (19) | 0/5 (0) | A versus D; p = 0.0072, A versus E; p = 0.0079 |
| Rituximab (%) | 1/4 (25) | 3/7 (43) | 6/12 (50) | 5/16 (31) | 0/5 (0) | C versus D; p = 0.0441 |
| Azathioprine (%) | 0/4 (0) | 1/7 (14) | 0/12 (0) | 1/16 (6) | 0/5 (0) | NS |
| Mitoxandrone (%) | 1/4 (25) | 0/7 (0) | 1/12 (8) | 1/16 (6) | 0/5 (0) | NS |
| Clinical response to treatment strategies | ||||||
| EDSS after acute treatment, mean (± SD) <3 months from disease initiation | 2.875 (0.8539) | 2.214 (1.468) | 2.542 (0.8649) | 2.375 (1.466) | 1.3 (0.4472) | A versus E; p = 0.0238, C versus E; p = 0.0134 |
| EDSS at last follow up, mean (± SD) | 3.75 (4.193) | 2.071 (1.170) | 2.5 (1.523) | 2.281 (1.653) | 1.2 (0.4472) | C versus E; p = 0.0498 |
NS = non-statistically significant difference among subgroup analysis for multiple comparisons for all of the p > 0.05.
CSF, cerebrospinal fluid; EDSS, Expanded Disability Status Scale; GD+, gadolinium enhancement; LETM, longitudinal extensive transverse myelitis; MRI, magnetic resonance imaging; MS, multiple sclerosis; PVWM, periventricular white matter; SD, standard deviation; T2w, T2 weighted; TDL, tumefactive demyelinating lesion; WBC, white blood cell.