Clinical features and immunological findings in patients with limbic encephalitis seen at the Hospital of the University of Pennsylvania (n = 17)
| Aza, azathioprine; CI, complete improvement; CTX, cyclophosphamide; F, female; IVIg, intravenous immunoglobulin; M, male; nCMAg, novel cell-membrane antigen; PI, partial improvement; PEX, plasma exchange; SCLC, small-cell lung cancer; VGKC, voltage -gated potassium channels; WBC, white blood cells. | |||||||
| *WBC (white cell count) per μl; normal value <4/μl. | |||||||
| †Prot (protein concentration) in mg/dl; normal value 16–46 mg/dl. | |||||||
| ‡Other: fluid attenuation inversion recovery or T2 abnormalities in regions other than medial temporal lobes. | |||||||
| Total patients (antigen) | Sex; age range (median), years | Other neurological features or symptoms | CSF findings, range (median) | Typical “limbic” MRI abnormality | Cancer | Immunotherapy other than corticosteroids | Neurological outcome |
| Hu, n = 4; Ma2, n = 1; atypical, n = 1 | 5M, 1F; 28–65 (63) | Encephalomyelitis, n = 2; sensory neuronopathy, n = 3; 2 cerebellar/or brainstem, n = 2 | *WBC 0–81 (3); †Prot 74–150 (79) | Typical, n = 4; other, n = 2‡ | SCLC, n = 4; testicular, n = 1; thyroid, n = 1 | IVIg, n = 1; CTX, n = 2;IVIg+PEX +Rituximab+Aza, n = 1 | PI (Ma2), n = 1; stable (Hu), n = 1;died, n = 4 |
| nCMAg, n = 5 | 5F; 24–65 (44) | Diffuse encephalitis, n = 2; decreased level of consciousness and hypoventilation, n = 1; chorea/dystonia, n = 1 | WBC 15–49 (30); Prot 18–97 (67) | ‡Other, n = 4; normal, n = 1 | Teratoma, n = 3 (ovary, n = 2; thymus, n = 1); cancer of thymus, n = 1; no tumour, n = 1 | IVIg, n = 1; PEX, n = 1;IVIg+PEX, n = 1 | CI (n = 4, teratoma, n = 3, cancer of thymus, n = 1); deteriorated (no tumour), n = 1 |
| VGKC, n = 3 | 2M, 1F; 38–60 (58) | Peripheral nerve hyperexcitability, n = 1 | WBC 0–4 (2); Prot 45–79 (63) | Typical, n = 3 | Prostate, n = 1 | IVIg, n = 2 | CI, n = 2; stable, n = 1 |
| No antibodies, n = 3 | 1M, 2F; 28–60 (40) | Multiple (cerebellar myelopathy neuropathy), n = 1 | WBC 2–119 (10); Prot 32–132 (47) | Typical, n = 2; other, n = 1 | Prostate, n = 1 | Aza, n = 1 | CI, n = 3 |