Anti-NMDA receptor encephalitis causes
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; AE Dheeraj Makkar, M.D.[2]
Overview
In anti-NMDA receptor encephalitis, autoantibodies targeting the GluN1 subunit of the NMDA receptor are present. These antibodies lead to internalization of NMDA receptors, disrupting synaptic plasticity and causing a range of neurological and psychiatric symptoms.
Causes
| Antibodies | Antigens | Associated symptoms | Tumors |
|---|---|---|---|
| Anti-Hu (ANNA-1) | HuD | Encephalomyelitis, limbic encephalitis, cerebellar degeneration, brain stem encephalitis, multi-segmental myelitis, sensory neuronopathy, sensory motor neuropathy, autonomic neuropathy | Lung cancer (85%), mostly SCLC, neuroblastoma, prostate carcinoma |
| Anti-Yo (PCA-1) | CDR2, CDR62 | Paraneoplastic cerebellar degeneration | Ovarian, breast cancer |
| Anti-CV2(CRMP5) | CRMP5 | Encephalomyelitis, polyneuropathy, optic neuritis, limbic encephalitis, choreatic syndromes, cerebellar degeneration | SCLC, thymoma |
| Anti-Ta/Ma2 | MA-proteins | Limbic encephalitis, rhombencephalitis, male>>female | Testicular cancer |
| Anti-Ri (ANNA-2) | NOVA-1 | Opsoclonus–myoclonus syndrome, rhombencephalitis, cerebellar degeneration, myelitis, jaw dystonia, laryngospasm | Breast, ovarian carcinoma, SCLC |
| Anti-amphiphysin | Amphiphysin | Stiff-person syndrome, limbic encephalitis, rhombencephalitis, cerebellar degeneration, polyneuropathy | Breast cancer, SCLC |
| Anti-recoverin | Recoverin | Retinopathy | SCLC |
| Anti-SOX-1 (AGNA) | SOX-1 | Non-syndrome-specific | Sensitivity 67%, specificity 95% for SCLC in LEMS |
| Antibodies | Antigens | Associated symptoms | Tumors |
|---|---|---|---|
| Anti-Tr (PCA-Tr) | DNER | Cerebellar degeneration | Hodgkin lymphoma, non-Hodgkin lymphoma |
| Anti-Zic4 | ZIC1-4 | Cerebellar degeneration | SCLC |
| PCA-2 | 280 kD | Encephalitis, Lambert–Eaton myasthenic syndrome, polyneuropathy | SCLC |
| ANNA-3 | 170 kD | Neuropathy, cerebellar degeneration, limbic encephalitis | SCLC |
SCLC: Small cell lung carcinoma; LEMS: Lambert–Eaton myasthenic syndrome (LEMS) DNER: delta/notch-like epidermal growth factor-related receptor
References
- ↑ Gresa-Arribas N, Titulaer MJ, Torrents A, Aguilar E, McCracken L, Leypoldt F; et al. (2014). "Antibody titres at diagnosis and during follow-up of anti-NMDA receptor encephalitis: a retrospective study". Lancet Neurol. 13 (2): 167–77. doi:10.1016/S1474-4422(13)70282-5. PMC 4006368. PMID 24360484.