Table 1.
Clinical and demographic information of the study cohort
| Species/Sample types | Sex/Age [years] | Clinical presentation | Prior immuno suppression |
Time to biopsy/death (autopsy) [months] | Site of brain biopsy/autopsy | Treatment | Disease duration/Overall outcome | Neuropathological phenotype |
|---|---|---|---|---|---|---|---|---|
| Canine/Autopsy | F/5 | NME, seizures, ataxia | No | 3 days | Brain autopsy | Antiepileptic | 3 days/Death | Acute necrotizing (severe; GM, WM) |
| Human/Biopsy | M/37 | MEM, ataxiaa | No | 3 (current episode) | Right temporal | Steroids, MMF | 7 years (current episode)/Normal | Lymphocytic (severe; GM, WM, Figure 7j, o) |
| Human/Biopsy | F/58 | MEM, tremor, ataxia | No | 3 | Right frontal | Steroids, MTX, IVIg, MMF |
10 months/Initial improvement, remaining behavioral and walking difficulties (mRS4); long-term follow-up N/A |
Lymphocytic (mild; GM, WM) |
| Human/Biopsy | M/53 | MEM, tremor | No | 4 | Right frontal | Steroids, MMF | 9 years/Improved but persistent tremor (mRS2) | Lymphocytic (moderate; GM, WM, Fig. 8a–o) |
| Human/Biopsy | F/29 | MEM, optic disk edema, tremor | No | 5 | Right frontal | Steroids, MMF | 2 years/Initially improved; after 2nd relapse persistent vertigo and hearing loss (mRS2) | Mixed granulomatous-like/lymphocytic (mild; GM, WM, Fig. 6a–d, u–x, Fig. 7d–i) |
| Human/Biopsy | F/42 | Tremor, encephalitis, seizures | No | 5 | Left temporal & meninges | Steroids, MMF | 11 years/Normal | Lymphocytic (moderate; GM, WM) |
| Human/Autopsy | F/54 | MEM, vertigo, behavioral changes, disorientation, hearing loss, aphasia, paraparesis, pallhypesthesia, visual loss, optic disc edema | Yes; steroids | 6 | Brain and spinal cord autopsy | Steroids | 6 months/Death | Granulomatous (severe; GM, WM) |
| Human/Autopsy | F/75 | ME, hallucinations, dysarthria, rigor, tetra-ataxia | Yes; steroids | 8 | Brain autopsy | Steroids | 8 months/Death | Lymphocytic (moderate; GM, WM) |
| Human/Biopsy | M/51 | MEM, tremor | No | 8 | Right frontal | Steroids, MMF | 9 years/Initial improvement; persistent myelopathy (mRS4) | Lymphocytic (moderate; GM, WM, Fig. 6i–t, Fig. 7m) |
| Human/Biopsy | M/36 | MEM with optic disk edema | Yes; steroids | 9 | Right frontal | Steroids, AZA | 11 years/Normal | Lymphocytic (severe; GM, WM, Fig. 6h; Fig. 7b, c) |
| Human/Biopsy | F/58 | MEM, tremor, optic disk edema | Yes; steroids, IFNβ | 9 | Left frontal | Steroids, MMF, changed for AZA after relapse | 3 years/Some tandem gait unsteadiness (mRS1) | Lymphocytic (moderate; WM, Fig. 7k, l, n) |
| Human/Biopsy | M/49 | Cognitive decline, headache, ambulation difficultiesb | No | 13 | Right temporal | Steroids, RTX | N/A | Lymphocytic (moderate; GM, WM, Fig. 6e–g) |
No co-existing anti-neuronal and anti-glial autoantibodies were detected
AZA azathioprine, F female, Fig. figure, GM gray matter, IFNβ interferon β, IVIg intravenous immunoglobulin, M male, ME meningoencephalitis, MEM meningoencephalomyelitis, MMF mycophenolate mofetil, mRS modified Rankin Score, MTX methotrexate, N/A not available, NME necrotizing meningoencephalitis, RTX Rituximab, WM white matter
a1st episode with similar presentation 6 years prior that spontaneously improved
bThe patient presented with severe leukoencephalopathy in the MRI without gadolinium enhancement