Table 1 Clinical characteristics, investigation findings, treatment and outcome of antibody-positive and antibody-negative AE patients
Characteristic | Clinically diagnosed NMDARE | Clinically diagnosed LE n (%) | |||
|---|---|---|---|---|---|
Total N (%) | NMDAR-Ab positive n (%) | NMDAR-Ab negativea n (%) | NMDAR-Ab positive Vs negative p-value | ||
Patients | 65 (100) | 29 (20.4) | 36 (25.4) | 6 (4.2) | |
Mean age (SD) | 25.6 (20.3) | 19.3 (11.9) | 30.7 (24.1) | 0.01 | 47.2 (17.5) |
Females | 42 (64.6) | 22 (75.9) | 20 (55.5) | 0.09 | 5 (83.3) |
Clinical manifestations | |||||
Decreased level of consciousness | 58 (89.2) | 23 (79.3) | 35 (97.2) | 0.01 | 4 (66.6) |
Abnormal (psychiatric) behaviour or cognitive dysfunction | 62 (95.3) | 27 (93.1) | 35 (97.2) | 0.43 | 6 (100) |
Amnesia/working memory deficits | 28 (43.0) | 9 (31.0) | 19 (52.8) | 0.08 | 3 (50) |
Confusion/disorientation in time, place or person | 56 (86.1) | 24 (82.8) | 32 (88.9) | 0.48 | 6 (100) |
Visual hallucinations | 19 (29.2) | 9 (31.0) | 10 (27.8) | 0.77 | 1 (16.6) |
Auditory hallucinations | 15 (23.1) | 8 (27.6) | 7 (19.4) | 0.44 | 0 |
Insomnia | 24 (36.9) | 12 (41.4) | 12 (33.3) | 0.50 | 3 (50.0) |
Agitation | 36 (55.4) | 19 (65.5) | 17 (47.2) | 0.14 | 5 (83.3) |
Catatonia | 23 (35.4) | 9 (31.0) | 14 (38.9) | 0.51 | 0 |
Obsessive thoughts or actions | 13 (20.0) | 6 (20.7) | 7 (19.4) | 0.90 | 0 |
Speech dysfunction | 30 (46.1) | 7 (24.1) | 23 (63.9) | 0.001 | 0 |
Seizures | 53 (81.5) | 25 (86.2) | 28 ((77.7) | 0.38 | 6 (100) |
Focal | 32 (49.2) | 15 (51.7) | 17 (47.2) | 0.72 | 2 (33.3) |
Generalised | 34 (52.3) | 15 (51.7) | 19 (52.8) | 0.93 | 4 (66.6) |
Status epilepticus | 18 (27.7) | 9 (31.0) | 9 (25) | 0.59 | 1 (16.6) |
Faciobrachial dystonic seizures | 0 | 0 | 0 | 0 | 0 |
Movement disorders | 45 (69.2) | 17 (58.6) | 28 (77.7) | 0.09 | 0 |
Orofacial dyskinesia | 30 (46.2) | 11 (37.9) | 19 (52.8) | 0.23 | 0 |
Limb dyskinesia | 25 (38.2) | 10 (34.5) | 15 (41.7) | 0.55 | 0 |
Parkinsonism | 5 (7.7) | 1 (3.4) | 4 (11.1) | 0.25 | 0 |
Dysautonomia | 16 (24.6) | 6 (20.6) | 10 (27.7) | 0.51 | 0 |
Hyperhidrosis | 4 (6.2) | 2 (6.9) | 2 (5.6) | 0.83 | 0 |
Hypersalivation | 9 (13.8) | 4 (13.8) | 5 (13.9) | 0.99 | 0 |
Fluctuating heart rate | 6 (9.2) | 2 (6.9) | 2 (5.6) | 0.56 | 0 |
Fluctuating blood pressure | 4 (6.2) | 2 (6.9) | 2 (5.6) | 0.83 | 0 |
Central hypoventilation | 0 | 0 | 0 | 0 | |
Tumours | 0 | 0 | 0 | 0 | |
Investigations | |||||
CSF protein elevated (number/of available data) | 21/57 (36.8) | 7/24 (29.2) | 14/33 (42.2) | 0.31 | 3/5 (60.0) |
CSF pleocytosis (number/of available data) | 22/58 (37.9) | 10/26 (38.5) | 12/32 (37.5) | 0.94 | 5/5 (100.0) |
Abnormal EEG (number/of available data) | 46/56 (82.1) | 14/22 (63.6) | 32/34 (94.1) | 0.003 | 2/4 (50.0) |
Abnormal MRI (number/of available data) | 13/40 (32.5) | 4/16 (25.0) | 9/24 (37.5) | 0.41 | 1/3 (33.3) |
Treatment | |||||
Intravenous methylprednisolone | 41 (63.0) | 20 (68.9) | 21 (58.3) | 0.38 | 6 (100) |
Intravenous immunoglobulins | 34 (52.3) | 17 (58.6) | 17 (47.2) | 0.36 | 2 (33.3) |
Plasmapheresis | 35 (53.8) | 19 (65.5) | 16 (44.4) | 0.09 | 0 |
Rituximab | 6 (9.2) | 4 (13.8) | 2 (5.5) | 0.25 | 0 |
Mycophenolate mofetil | 2 (3.0) | 1 (3.4) | 1 (2.7) | 0.25 | 0 |
Anti-epileptic medication | 37 (56.9) | 16 (55.1) | 21 (58.3) | 0.79 | 4 (66.6) |
Outcome at one-year review | |||||
Recovery to mRS < 3 (number/of available data) | 41/60 (68.3) | 23/29 (79.3) | 18/31 (58.0) | 0.07 | 3/5 (60.0) |
Deaths | 14/60 (23.3) | 4 (13.8) | 10 (27.8) | 0.17 | 2 (33.3) |