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024 7 _ |a 10.3389/fneur.2023.1193015
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037 _ _ |a DZNE-2023-00706
041 _ _ |a English
082 _ _ |a 610
100 1 _ |a Brauchle, Felix
|b 0
245 _ _ |a Clinical 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.
260 _ _ |a Lausanne
|c 2023
|b Frontiers Research Foundation
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520 _ _ |a 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.
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650 _ 7 |a MRZ reaction
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650 _ 7 |a autoimmue disease
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650 _ 7 |a multiple sclerosis
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650 _ 7 |a neuroinflammation
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650 _ 7 |a polyspecific intrathecal immune response
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700 1 _ |a Rapp, Daniel
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700 1 _ |a Senel, Makbule
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700 1 _ |a Huss, André
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700 1 _ |a Dreyhaupt, Jens
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700 1 _ |a Klose, Veronika
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700 1 _ |a Süße, Marie
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700 1 _ |a Stürner, Klarissa Hanja
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700 1 _ |a Leypoldt, Frank
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700 1 _ |a Tumani, Hayrettin
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700 1 _ |a Lewerenz, Jan
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773 _ _ |a 10.3389/fneur.2023.1193015
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856 4 _ |u https://www.frontiersin.org/articles/10.3389/fneur.2023.1193015/full
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