Table 1. Clinical, investigations, treatment and outcome details of ADEM, anti-NMDAR encephalitis and enteroviral encephalitis cohorts.
Clinical syndrome (n) | ADEM (16) | Anti-NMDAR-E (11) | EVE (16) | Control (20) |
---|---|---|---|---|
Gender, female/male | 4/12 | 5/6 | 5/11 | 9F, 11M |
Age, median (range), y | 6.3 (2–13.3) | 6 (2.6–14) | 2.7(1.3–9.5) * | 4.9 (0.3–14)# |
ICU admission (n), duration (d) | 2/16 (12%) (3–4 d) | 4/11 (36%) (21–26 d) | 7/16 (44%) (1–7 d) | NA |
Timing of CSF from onset of acute neurological symptoms, median (range), days | 4 (1–21) | 16 (4–24) * | 3 (1–12) | NA |
CSF Pleocytosis (>5x106 cells/L) | 13 (81%) | 8 (72%) | 11 (68%) | 0 |
CSF Pleocytosis, median (range), x106 /L | 33 (0–175) | 15 (0–170) | 43.5 (0–86) | 0 (0–3) |
CSF neutrophils, median (range), x106 /L | 4 (0–25) | 0 (0–3) | 2 (0–10) | 0 (0–1) |
CSF protein, median (range), mg/dl | 0.36 (0.15–0.7) | 0.22 (0.1–0.77) | 0.26 (0.1–0.31) | NA |
Neopterin (Elevated> 29 nmol/L), n | 13 (81%) | 11(100%) | 16 (100%) | 1(6PTPS deficiency) |
Oligoclonal IgG bands (positive), n | 2/15 (13%) | 4/11 (36%) | 1/10 (10%) | NA |
MRI Brain abnormal, n | 16/16 (100%) | 3/11 (27%) | 14/15 (93%) | - |
MRI spine abnormal | 9/12 (75%) | Not done | 7/10 (70%) | |
Immunotherapy | 15/16 (93%) [Steroids (n = 15), IVIG (n = 1), mycophenolate (n = 2)] | 10/11 (90%) [Steroids (n = 10), IVIG (n = 8), rituximab (n = 3)] | 8/16 (50%) [Steroids (n = 6), IVIG (n = 6) | - |
Relapse | 3/16 (18%) | 3/11(27%) | 0/16 (0%) | - |
Duration of FU, median (range), mo | 16 (1–54) | 27 (11–66) | 6 (1–30) | - |
FU outcome (MRS score) | ||||
No disability (0–1) | 10/16 (62%) | 6/11 (54%) | 11/16 (68%) | |
Mild disability (2) | 4/16 (25%) | 2/11 (18%) | 1/16 (6%) | |
Moderate disability (3) | 2/16 (12%) | 3/11 (27%) | 1/16 (6%) |
*Highlighted results represent variables with P<0.05 in the Kruskal Wallis test, see results section for more details
# age at the time of CSF sampling
Abbreviations: ADEM, acute disseminated encephalomyelitis; Anti-NMDAR E, anti-N-Methyl D-Aspartate receptor encephalitis; EVE, entero viral encephalitis; y, years; d, day; mo, months: CSF, cerebrospinal fluid; MRI, magnetic resonance imaging; ICU, intensive care unit admission; MRS, modified Rankin scale; FU, follow up; IVIG, immunoglobulins