Clinical features and immunological findings in patients with limbic encephalitis seen at other institutions (n = 22)
Total patients(antigen) | Sex; age range(median) | Other neurological features or symptoms | CSF finding range (median) | Typical “limbic”MRI abnormality | Cancer | Immunotherapy other than corticosteroids | Neurological outcome |
---|---|---|---|---|---|---|---|
CI, complete improvement; CTX, cyclophosphamide; F, female; IVIg, intravenous immunoglobulin; M, male; NA, not available; nCMAg, novel cell-membrane antigen; PEX, plasma exchange; PI, partial improvement; SCLC, small-cell lung cancer; VGKC, voltage-gated potassium channels. | |||||||
*WBC (white blood count) per μl; normal value <4/μl. | |||||||
†Prot (protein concentration) in mg/dl; normal value 16–46 mg/dl. | |||||||
Hu, n = 3; Ma2, n = 5 | 6M, 2F;23–67 (36) | Encephalomyelitis, n = 1; brainstem, n = 2; hypothalamic, n = 1 | *WBC 2–20 (18); †Prot 30–747 (80) | Typical, n = 4; other, n = 3; NA, n = 1 | SCLC, n = 3; non-SCLC, n = 1; testicular, n = 4 | PEX+IVIg, n = 1; PEX, n = 1; IVIg, n = 3 | PI (Ma2), n = 1; CI (Hu), n = 1; stable (Ma2), n = 1; died (Ma2, n = 2; Hu, n = 1), n = 3; no follow-up, n = 2 |
nCMAg, n = 12 | 2M, 10F;3–40 (29) | Decreased level of consciousness and hypoventilation, n = 9; dystonia/choreoathetosis, n = 6; stiff-person, n = 1 | WBC 7–189 (20); Prot 30–92 (50) | Typical, n = 6; other, n = 4; normal, n = 2 | Thymoma, n = 1; teratoma of the ovary, n = 8; no tumour, n = 1; not known, n = 2 | PEX, n = 3; IVIg, n = 2; PEX+IVIg, n = 2; PEX+IVIg+CTX, n = 2 | PI (ovarian teratoma, n = 3; thymoma, n = 1; no tumour, n = 1), n = 5; CI (ovarian teratoma), n = 3; died (ovarian teratoma), n = 2; no follow-up, n = 2 |
VGKC, n = 2 | 2M; 58 and 59 | – | WBC 0–2; Prot normal | Typical, n = 2 | No cancer | PEX, n = 1; IVIg, n = 1 | CI (relapsing syndrome, n = 1), n = 2 |