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@ARTICLE{vonZedtwitz:281364,
author = {von Zedtwitz, Katharina and Weiser, Judith and Dressle,
Raphael J and Maier, Simon J and Feige, Bernd and Nickel,
Kathrin and Venhoff, Nils and Domschke, Katharina and
Brumberg, Joachim and Rauer, Sebastian and Tebartz van Elst,
Ludger and Hannibal, Luciana and Prüss, Harald and Rau,
Alexander and Endres, Dominique},
title = {{C}ase {R}eport: {A}cute polymorphic psychosis and
{NMDA}-{R} {I}g{G} antibodies in serum: a follow-up case
study.},
journal = {Frontiers in immunology},
volume = {16},
issn = {1664-3224},
address = {Lausanne},
publisher = {Frontiers Media},
reportid = {DZNE-2025-01111},
pages = {1630357},
year = {2025},
abstract = {Anti-N-methyl-D-aspartate receptor (NMDA-R) encephalitis is
a neuropsychiatric disorder with additional psychiatric
features caused by NMDA-R immunoglobulin G (IgG) antibodies
in cerebrospinal fluid (CSF). This report presents the
follow-up of a patient in whom we assumed mild NMDA-R
encephalitis in the first psychotic episode.A patient with a
prior episode of an acute polymorphic psychotic syndrome
relapsed five and a half years later following a severe
COVID-19 infection. Serum NMDA-R antibodies were again
detected with a titer of max. 1:320 using fixed-cell-based
assays, but conventional magnetic resonance imaging (MRI),
electroencephalography (EEG), and CSF findings were largely
normal. NMDA-R antibody levels in serum decreased to 1:80
after approximately one month without immunotherapy.
[18F]fluorodeoxyglucose positron emission tomography
(FDG-PET) still revealed pronounced metabolism of the
association cortices (clearly more pronounced in the first
episode with an encephalitis-like pattern at that time).
Advanced MRI analyses including diffusion microstructure
imaging (DMI) showed frontal and thalamic microstructural
alterations compatible with edematization (but also far less
accentuated than in the first episode). Further advanced
antibody tests of CSF (approx. 1 month after symptom onset)
using a live-cell-based and different tissue-based assays
were negative for NMDA-R IgG antibodies. Research mass
spectrometry of the CSF identified
neurotransmitter-precursor shortages, increased turnover of
tryptophan into quinolinic acid, and low-glucose/lactate
levels. Immunotherapy (performed after the initial
assumption of an autoimmune cause) with steroids led to
clinical improvement of residual symptoms. After
approximately three months, NMDA-R IgG serum antibodies were
no longer detectable; however, FDG-PET/DMI follow-up
revealed no relevant changes.The international consensus
criteria for a probable/definite diagnosis of NMDA-R
encephalitis or autoimmune psychosis were not fulfilled,
especially as no NMDA-R IgG antibodies were identified in
CSF using different antibody assays and EEG/CSF routine
findings were inconspicuous. NMDA-R encephalitis was
therefore not diagnosed (as initially suspected).
Independent of the NMDA-R IgG antibodies, there were
possible signs of an autoimmune process. For a better
understanding of similar patients, multimodal diagnostic
approaches including complementary antibody tests could be
promising.},
keywords = {Humans / Anti-N-Methyl-D-Aspartate Receptor Encephalitis:
immunology / Anti-N-Methyl-D-Aspartate Receptor
Encephalitis: diagnosis / Anti-N-Methyl-D-Aspartate Receptor
Encephalitis: blood / Autoantibodies: blood /
Autoantibodies: immunology / COVID-19: complications /
COVID-19: immunology / Follow-Up Studies / Immunoglobulin G:
blood / Immunoglobulin G: immunology / Magnetic Resonance
Imaging / Positron-Emission Tomography / Psychotic
Disorders: immunology / Psychotic Disorders: blood /
Psychotic Disorders: diagnosis / Psychotic Disorders:
etiology / Receptors, N-Methyl-D-Aspartate: immunology /
autoimmune (Other) / autoimmune encephalitis (Other) / brain
(Other) / immunotherapy (Other) / inflammation (Other) /
Autoantibodies (NLM Chemicals) / Immunoglobulin G (NLM
Chemicals) / Receptors, N-Methyl-D-Aspartate (NLM
Chemicals)},
cin = {AG Prüß},
ddc = {610},
cid = {I:(DE-2719)1810003},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:40927716},
pmc = {pmc:PMC12414783},
doi = {10.3389/fimmu.2025.1630357},
url = {https://pub.dzne.de/record/281364},
}