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@ARTICLE{Brauchle:258940,
author = {Brauchle, Felix and Rapp, Daniel and Senel, Makbule and
Huss, André and Dreyhaupt, Jens and Klose, Veronika and
Süße, Marie and Stürner, Klarissa Hanja and Leypoldt,
Frank and Tumani, Hayrettin and Lewerenz, Jan},
title = {{C}linical associations and characteristics of the
polyspecific intrathecal immune response in elderly patients
with non-multiple sclerosis chronic autoimmune-inflammatory
neurological diseases - a retrospective cross-sectional
study.},
journal = {Frontiers in neurology},
volume = {14},
issn = {1664-2295},
address = {Lausanne},
publisher = {Frontiers Research Foundation},
reportid = {DZNE-2023-00706},
pages = {1193015},
year = {2023},
abstract = {The polyspecific intrathecal immune response (PSIIR), aka
MRZ reaction (M = measles, R = rubella, Z = zoster,
optionally Herpes simplex virus, HSV) is defined as
intrathecal immunoglobulin synthesis (IIS) for two or more
unrelated viruses. Although an established cerebrospinal
fluid (CSF) biomarker for multiple sclerosis (MS), a chronic
autoimmune-inflammatory neurological disease (CAIND) of the
central nervous system (CNS) usually starting in young
adulthood, the full spectrum of CAINDs with a positive PSIIR
remains ill defined.In this retrospective, cross-sectional
study, patients with CSF-positive oligoclonal bands (OCB)
and - to enrich for non-MS diagnoses - aged ≥50 years were
enrolled.Of 415 with PSIIR testing results (MRZ, HSV
optional), 76 were PSIIR-positive. Of these, 25 $(33\%)$ did
not meet the diagnostic criteria for MS spectrum diseases
(MS-S) comprising clinically or radiologically isolated
syndrome (CIS/RIS) or MS. PSIIR-positive non-MS-S phenotypes
were heterogenous with CNS, peripheral nerve and motor
neuron involvement and often defied unequivocal diagnostic
classification. A rating by neuroimmunology experts
suggested non-MS CAINDs in 16/25 $(64\%).$ Long-term
follow-up available in 13 always showed a chronically
progressive course. Four of five responded to immunotherapy.
Compared to MS-S patients, non-MS CAIND patients showed less
frequent CNS regions with demyelination $(25\%$ vs. $75\%)$
and quantitative IgG IIS $(31\%$ vs. $81\%).$ MRZ-specific
IIS did not differ between both groups, while additional
HSV-specific IIS was characteristic for non-MS CAIND
patients.In conclusion, PSIIR positivity occurs frequently
in non-MS-S patients ≥50 years. Although sometimes
apparently coincidental, the PSIIR seems to represent a
suitable biomarker for previously unnoticed chronic
neurologic autoimmunities, which require further
characterization.},
keywords = {MRZ reaction (Other) / autoimmue disease (Other) / multiple
sclerosis (Other) / neuroinflammation (Other) / polyspecific
intrathecal immune response (Other)},
cin = {AG Zhan},
ddc = {610},
cid = {I:(DE-2719)1910005},
pnm = {354 - Disease Prevention and Healthy Aging (POF4-354)},
pid = {G:(DE-HGF)POF4-354},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:37396770},
pmc = {pmc:PMC10311206},
doi = {10.3389/fneur.2023.1193015},
url = {https://pub.dzne.de/record/258940},
}