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NMDAR-AK N-Methyl-D-Aspartat-Rezeptor - Antikörper Psychose, epileptische Anfälle, Status epilepticus Heilung durch Immunsuppression: Corticoide, IVIG, Plasmapherese
Lgi-1 Leucin-Glioma-rich-1-Rezeptor Epilepsie, therapieresistent, irreversible Hippocampus-Sklerose
Caspr2 Contactin-associated-Protein-Receptor 2 Epilepsie, Demenz
Anti-Hu paraneoplastisches Syndrom ohne Tumor
Antikörper - vermittelte Enzephalitis (1)
Antikörper Patienten Alter Männer / Frauen Klinik Syndrom MR Tumoren IgG-Klasse Antikörper-Wirkung
NMDAR (>1500) 21 yr (2 mo–85 yr) 1:4 Children: seizures, dyskinesias; adults: behavioral changes, psychiatric symptoms NMDAR encephalitis Normal findings (70%) or nonspecific changes Varies with age and sex; ovarian teratoma in women 18–45 yr old (58%) IgG1 Internalization of NMDAR, disruption of NMDAR interaction with ephrin-B2 receptor
AMPAR (80) 56 yr (23–81) 1:2.3 Confusion, memory loss; in rare cases, psychiatric symptoms Limbic encephalitis Increased signal in medial temporal lobes (67%) SCLC, thymoma, or breast cancer (56%) IgG1 Internalization of AMPARs
GABABR (80) 61 yr (16–77) 1.5:1 Seizures, memory loss,confusion Limbic encephalitis, prominent seizures Increased signal in medial temporal lobes (45%) SCLC (50%) IgG1 Blocking of agonist effect of baclofen on GABABR
LGI1 (400) 64 yr (31–84) 2:1 Memory loss, faciobrachial dystonic seizures, hyponatremia Limbic encephalitis Increased signal in medial temporal lobes (83%) Thymoma (<5%) IgG4 Inhibition of LGI1 interaction with ADAM22 and ADAM23; decrease in postsynaptic AMPAR
CASPR2 (120) 66 yr (25–77) 9:1 Memory loss, insomnia, dysautonomia, ataxia, peripheral-nerve hyperexcitability, neuropathic pain Limbic encephalitis Increased signal in medial temporal lobes (67%) Varies with the syndrome (<5% overall) IgG4 Alteration of gephyrin clusters in inhibitory synapses
mGluR5 (11) 29 yr (6–75) 1.5:1 Confusion, psychiatric symptoms Encephalitis Normal findings in 5 of 11 patients Hodgkin’s lymphoma in 6 of 11 patients IgG1 Decrease in density of surface mGluR5
D2R (25) 6 yr (2–15) 1:1 Parkinsonism, dystonia, psychiatric symptoms Basal ganglia encephalitis Increased signal in basal ganglia (50%) No associated cancer Unknown Receptor internalization and decrease in D2R surface density
DPPX (45) 52 yr (13–76) 2.3:1 Confusion, diarrhea, weight loss Encephalitis, myoclonus, tremors, hyperekplexia Normal findings or nonspecific changes (100%) B-cell neoplasms (<10%) IgG4 Decrease in density of surface DPPX and Kv4.2
GABAAR (70) 40 yr (2 mo–88 yr) 1:1 Seizures, confusion, behavioral changes Encephalitis, frequent status epilepticus Cortical and subcortical FLAIR signal abnormalities involving two or more brain regions (77%) Thymoma (27%) IgG1 Selective reduction of GABAAR at synapses
Neurexin-3α (6) 44 yr (23–57) 2:4 Confusion, seizures Encephalitis Normal findings in 4 of 6 patients No associated cancer Unknown Decrease in density of surface neurexin-3α and total number of synapses in neurons undergoing development
paraneoplasische Neuropathien
Quellen 1.) Dalmau J, Graus F:
Antibody-Mediated Encephalitis.
N Engl J Med 2018;378:840-51.
DOI: 10.1056/NEJMra1708712

Links GENERATE – GErman NEtwork for REsearch on AuToimmune Encephalitis

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Neurologie Neurologie, Psychiatrie Erkrankungen des Immunsystems

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