Table 1.
Demographic characteristics and clinical presentation of 144 patients referred for possible HE/SREAT diagnosis
| Autoimmune CNS disorder (n = 39) | Alternative diagnosis (n = 105) | Total (n = 144) | P value | |
|---|---|---|---|---|
| Female | 24 (61.5) | 79 (75.2) | 103 (71.5) | 0.15 |
| Median age (years) at onset (range) | 46.0 (13–87) | 44.0 (10–81) | 44.5 (10–87) | 0.08 |
| Median duration (months) of symptoms (range) | 16 (2–187) | 30 (1–414) | 25 (1–414) | 0.02 |
| Sub-acute onset (<3 months) | 32 (82.1) | 29 (27.6) | 61 (42.4) | <0.001 |
| Fluctuating course | 23 (59) | 46 (43.8) | 69 (47.9) | 0.13 |
| History of autoimmune thyroid disease | 24 (61.5) | 78 (74.3) | 102 (70.8) | 0.15 |
| Co-existing autoimmune disorder | 13 (33.3) | 17 (16.2) | 30 (20.8) | 0.04 |
| Past history of neoplasm | 4 (10.3) | 5 (4.8) | 9 (6.3) | 0.25 |
| Family history of autoimmune disorder | 22 (56.4) | 39 (37.1) | 61 (42.4) | 0.06 |
| Prior immunotherapy | 35 (89.7) | 79 (75.2) | 114 (79.2) | 0.07 |
| Reported immunotherapy response | 33 (94.3) | 29 (36.3) | 62 (53.9) | <0.001 |
| Clinical presentation | ||||
| Cognitive complaint | 37 (94.9) | 94 (89.5) | 131 (91) | 0.51 |
| Seizures | 10 (25.6) | 8 (7.6) | 18 (12.5) | 0.008 |
| Stroke-like episodes | 8 (20.5) | 5 (4.8) | 13 (9) | 0.007 |
| Language deficit | 11 (28.2) | 13 (12.4) | 24 (16.7) | 0.04 |
| Ataxia | 6 (15.4) | 4 (3.8) | 10 (6.9) | 0.02 |
| Apraxia | 3 (7.7) | 7 (6.7) | 10 (6.9) | >0.99 |
| Motor or sensory deficits | 15 (38.5) | 22 (21) | 37 (25.7) | 0.05 |
| Tremor | 8 (20.5) | 11 (10.5) | 19 (13.2) | 0.16 |
| Myoclonus | 2 (5.1) | 5 (4.8) | 7 (4.9) | >0.99 |
| Hypersomnolence | 5 (12.8) | 17 (16.2) | 22 (15.3) | 0.8 |
| Psychosis | 13 (33.3) | 21 (20) | 34 (23.6) | 0.12 |
| Headache | 9 (23.1) | 27 (25.7) | 36 (25) | 0.83 |
| Depression | 5 (12.8) | 37 (35.2) | 42 (29.2) | 0.008 |
| Anxiety | 1 (2.6) | 25 (23.8) | 26 (18.1) | 0.003 |
| Chronic pain | 2 (5.1) | 31 (29.5) | 33 (22.9) | 0.002 |
Categorical data provided as number (percentage). Bold values denote statistically significant results (P < 0.05).