Anti-NMDA receptor encephalitis causes

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 and the tumors producing them
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

Partially characterized onconeural antibodies (antigen not characterized or positive predictive value for tumor unknown)
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

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↑ 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.

[pmid24360484-1] 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.

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