Table 1.
Literature review of NMDA encephalitis and refractory cases with bortezomib.
| Age, gender, ethnicity | Time to diagnosis | Initial anti-NMDA serum/CSF titers | Clinical phenotype | Immunotherapy use and time to bortezomib treatment | References | |
|---|---|---|---|---|---|---|
| 1 | Early 30 s, female, African descent | Not reported | 1:1,000 (serum) at ~2 months | Acute agitation, hallucinations, catatonia, autonomic instability, tetraparesis | Steroids, PLEX, IVIg, rituximab, cyclophosphamide, bortezomib (~8 months). Resolution at ~11 months. | (12) |
| 2 | Early 20s, female, Caucasian | Not reported | 1:10 (serum) and 1:10 (CSF) at ~5 months | Behavioral changes, hallucinations, gait ataxia, central hypoventilation. Relapse 20 months later—gait ataxia, confusion, hallucinations, sexual disinhibition | PLEX, rituximab, steroids, IVIg, bortezomib (~29 months after initial onset) | |
| 3 | 22, female | Not reported | Not detectable in serum and 1:10 (CSF) | Catatonia, psychosis, agitation, autonomic dysfunction, seizures | Steroids, PLEX, rituximab, cyclophosphamide, bortezomib (~7.5 months) | (13) |
| 4 | 28, female | Not reported | 1:100 (serum) and 1:3.2 (CSF) | Anxiety, panic attacks, aggression, hypersomnia, seizures | PLEX, IVIg, rituximab, cyclophosphamide, bortezomib (~68 months) | |
| 5 | 19, female | Not reported | 1:320 (serum) and 1:32 (CSF) | Psychosis, cardia arrest, seizures | Steroids, PLEX, rituximab, bortezomib (~6 months) | |
| 6 | 22, female | Not reported | 1:10,000 (serum) and 1:320 (CSF | Psychosis, dissociative behavior, chorea, hyperkinesia, tachycardia, seizures | Steroids, PLEX, IVIg, rituximab, bortezomib (~3 months) | |
| 7 | 61, male | Not reported | 1:320 (serum) and 1:100 (CSF) | Vegetative state, seizures | Steroids, PLEX, IVIg, rituximab, bortezomib (~17 months) | |
| 8 | 26, female, Southeast Asian | 1 month | 1:2,500 (CSF) at diagnosis | Neuropsychiatric, aggression, hallucinations, insomnia, seizures | Steroids, rituximab, PLEX, IVIg, cyclophosphamide, tocilizumab, bortezomib (day 147). Discharged to rehabilitation clinic at day 313 | (14) |
| 9 | 22, female, Caucasian | Within first days of hospital admission | 1:400 (CSF) at diagnosis | Subacute psychosis, seizures, motor stereotypies | Steroids, IVIg, PLEX, rituximab, bortezomib (3 months). Discharged to rehabilitation clinic at ~25–30 days later | (15) |
| 10 | 20, female | First-line therapy initiated at 15 days | 1:640 (serum) at admission | Vegetative state, motor stereotypies, autonomic instability, central hypoventilation, seizures | Steroids, IVIg, PLEX, rituximab, tocilizumab, bortezomib (~9 months) | (16) |
| 11 | 28, female | First-line therapy initiated at 7 days | 1:1,280 (serum) and 1:640 (CSF) at admission | Vegetative state, motor stereotypies, central hypoventilation, sympathetic paroxysmal hyperactivity, seizures | Steroids, IVIg, rituximab, cyclophosphamide, tocilizumab, IL2, bortezomib (~12 months) | |
| 12 | 58, male | First line therapy initiated at 70 days | 1:160 (serum) and 1:160 (CSF) at admission | Vegetative state, motor stereotypies, rigidity, seizures | Steroids, IVIg, rituximab, tocilizumab, bortezomib (~5 months) | |
| 13 | 17, female | First line therapy initiated at 11 days | 1:320 (CSF) at admission | Vegetative state, motor stereotypies, rigidity, sympathetic paroxysmal hyperactivity, seizures | Steroids, IVIg, rituximab, tocilizumab, bortezomib (~5 months) | |
| 14 | 51, male | First line therapy initiated at 9 days | Not checked | Vegetative state, motor stereotypies, seizures | Steroids, IVIg, PLEX, rituximab, tocilizumab, bortezomib (~5 months) | |
| 15 | 35, female | Within first days of hospital admission | Positive in CSF and serum | Personality disorder, emotional lability, amnesia, seizures, paranoia, hallucinations | Steroids, IVIg, PLEX, rituximab, cyclophosphamide, bortezomib (3 cycles; ~357 days) | (11) |
| 16 | 49, female | Within first days of hospital admission | 1:250 (CSF) and ~1:750 (serum) | Headache, myalgia, behavioral changes, seizures, involuntary movements | PLEX, IVIg, cyclophosphamide, rituximab, bortezomib (~11 months) | |
| 17 | 18, female, African American | 6 days | 1:128 (CSF) at day 6 | Severe agitation, seizures, dystonia | Steroids, IVIg, PLEX, rituximab, bortezomib (day 40). Hospital discharge on day 70 | Current case |
NMDA, N-methyl-D-aspartate; CSF, cerebrospinal fluid; PLEX, plasma exchange; IVIg, intravenous immunoglobulin.