Table 1.
Characteristics and treatment response of patients treated with anakinra.
| Patient ID | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | 11 | 12 |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Age/sex | 29/F | 51/M | 54/M | 24/M | 56/M | 19/F | 18/F | 50/F | 74/M | 45/F | 56/M | 65/M |
| Clinical symptoms/signs | Gait disturbance, urinary retention, fecal inconsistency | Fever, altered mentality, memory impairment, seizure | Fever, altered mentality | Fever, Altered mentality, seizure, weakness in both legs, gaze palsy, hiccups, dysphagia | General weakness, altered mentality, memory impairment, Dysarthria | Fever, seizure | Fever, seizure | Depression, cognitive decline, dysarthria, dysphagia, gait disturbance, seizure | Fever, cognitive decline, memory impairment, gait disturbance | Altered mentality | Fever, altered mentality | Anxiety, cognitive decline, memory impairment, seizure, gait disturbance |
| Diagnosis | CAIR with PPMS | CAIR with GPA | CAIR with ADEM | ADEM | ADEM, chronic stage | NORSE | NORSE | Seronegative AE | Autoimmune meningitis | MTX‐necrotizing leukoencephalopathy | JBE | Sporadic CJD |
| Brain biopsy | CD68 (++): Moderate microglia infiltration | CD68 (+++): Diffuse microglia infiltration with chronic granulomatous and suppurative vasculitis | CD68 (++): Moderate microglia infiltration | N/A | LFB (−): Multifocal demyelination around vein | N/A | N/A | CD68 (+): Mild nonfoamy microglia with dominant CD8+ T cell infiltration | CD68 (+): mild nonfoamy microglia | N/A | N/A | RT‐QuIC: PrPsc (+) |
| CSF profile at worst | W8 (L8), Ptn47, IgG index 3.04 | W855 (P565, L145, O145), Ptn229, IgG index 0.87 | W2 (L2), Ptn237, IgG index 0.63 | W150 (P4, L146, O0), Ptn272 | W26 (P1, L25), Ptn 98 | W2 (L2), Ptn25, IgG index 0.41 | W2 (L2), Ptn 58 | W16 (P2, L9, O5), Ptn 97 | W240 (P158, L5, O77), Ptn 703, IgG index 0.64 | N/A | W389 (L389), Ptn 92 | W0, Ptn 90, IgG index 0.53 |
| MRI lesions | T2 lesion in bilat. Subcortical white matter, periventricular white matter, middle cerebellar peduncle, and cerebellum | T2 lesion in bilat. Medial temporal lobes and basal ganglia with leptomeningeal enhancement | Diffusion restriction and T2 lesion in bilat. Subcortical white matter with enhancement | Diffusion restriction and T2 lesion in bilat. Basal ganglia, periventricular white matter, midbrain, pons, and cerebellum with leptomeningeal enhancement | T1 high signal intensity in Rt. subcortical area, T2 lesion at bilat. Cerebral white matter | T2 lesion in bilat. Basal frontal lobes, parietotemporal lobes, insular cortex, medial occipital lobes, and posteromedial thalami. | Diffusion restriction and T2 lesion in bilat. Caudate nucleus and putamen. | T2 lesion in bilat. Parasagittal white matter, temporal area, and insula | Diffusion restriction at Rt. parahippocampal gyrus and corpus callosum. T2 lesion in bilat. Cerebral white matter and mesial temporal lobes with leptomeningeal enhancement | Diffusion restriction and T2 lesion in bilat. Fronto‐temporal lobes and middle cerebellar peduncle with multifocal enhancement | T2 lesion in bilat. Basal ganglia, thalami, and brainstem with diffuse enhancement | Diffusion restriction at pancortical regions |
| ImmunoTx before anakinra | Steroid, IVIg, RTX, TCZ, Tofacitinib | Steroid, IVIg, RTX, TCZ | Steroid, IVIg, RTX, TCZ | Steroid, IVIg | Steroid, IVIg, CTX, MMF | Steroid, IVIg, RTX, TCZ, Tofacitinib | Steroid, IVIg, RTX, TCZ | Steroid, IVIg, MMF, RTX, TCZ | Steroid, IVIg, RTX, TCZ, proleukin | Steroid | IVIg | IVIg (before diagnosis) |
| Duration of disease onset to anakinra | 4.5 years | 10 weeks | 4 weeks | 7 weeks | 8 months | 4 weeks | 5 weeks | 14 months | 12 months | 2 months | 11 months | 5 months |
| Response to anakinra | Very good | Very good | Good | Good | Unclear | Unclear | Unclear | Unclear | Unclear | Unclear | Unclear | Unclear |
| Duration of anakinra | 5 months | 14 days | 4 weeks | 1 week | 1 week | 6 weeks | 4 days | 2 weeks | 1 week | 2 weeks | 1 week | 1 week |
| mRS change by anakinra | 4➔2 | 5➔1 | 5➔4 | 5➔4 | 5➔5 | 5➔5 | 5➔5 | 5➔5 | 4➔4 | 5➔ 6 | 5➔5 | 5➔5 |
| CASE change by anakinra | 3➔1 | 12➔0 | 19➔8 | 9➔2 | 16➔16 | 20➔20 | 23➔23 | 18➔18 | 8➔9 | 17➔20 | 19➔19 | 17➔17 |
| Adverse events | None | None | None | None | None | Neutropenia | None | None | Confusion | None | None | None |
AE, autoimmune encephalitis; ADEM, acute disseminated encephalomyelitis; Bilat., bilateral; CASE, clinical assessment scale in autoimmune encephalitis; CAIR, cerebral autoinflammatory response; CJD, Creutzfeldt–Jakob disease; CSF, cerebrospinal fluid; F, female; GPA, granulomatosis with polyangiitis; IgG, immunoglobulin G; ImmunoTx, immunotherapy; IVIg, intravenous immunoglobulin; JBE, Japanese B encephalitis; L, lymphocytic cells; LFB, Luxol fast blue; M, male; MMF, mycophenolate mofetil; MRI, magnetic resonance imaging; mRS, modified Rankin Scale; MTX, methotrexate; N/A, not available; NORSE, new‐onset refractory status epilepticus; O, other cells; P, polymorphonuclear cells; PPMS, primary progressive multiple sclerosis; PrPsc, scrapie isoform of the prion protein; Ptn, protein; Rt., right; RT‐QuIC, the real‐time quaking‐induced conversion; RTX, rituximab; TCZ, tocilizumab.