Anti-NMDA receptor encephalitis causes: Difference between revisions

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__NOTOC__
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{{Anti-NMDA receptor encephalitis}}
{{Anti-NMDA receptor encephalitis}}
{{CMG}}
{{CMG}}; AE {{DMakkar}}


==Overview==
==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==
==Causes==
{| class="wikitable"
|+ 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
|}
{|class="wikitable"
|+ 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==
==References==
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[[Category:Rheumatology]]
[[Category:Rheumatology]]
[[Category:Disease]]
[[Category:Disease]]
 
<ref name="pmid24360484">{{cite journal| author=Gresa-Arribas N, Titulaer MJ, Torrents A, Aguilar E, McCracken L, Leypoldt F | display-authors=etal| title=Antibody titres at diagnosis and during follow-up of anti-NMDA receptor encephalitis: a retrospective study. | journal=Lancet Neurol | year= 2014 | volume= 13 | issue= 2 | pages= 167-77 | pmid=24360484 | doi=10.1016/S1474-4422(13)70282-5 | pmc=4006368 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24360484  }} </ref>
{{WH}}
{{WH}}
{{WS}}
{{WS}}

Latest revision as of 07:13, 13 May 2023

Anti-NMDA receptor encephalitis Microchapters

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Overview

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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 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

[1] Template:WH Template:WS

  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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