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000258940 1001_ $$aBrauchle, Felix$$b0
000258940 245__ $$aClinical associations and characteristics of the polyspecific intrathecal immune response in elderly patients with non-multiple sclerosis chronic autoimmune-inflammatory neurological diseases - a retrospective cross-sectional study.
000258940 260__ $$aLausanne$$bFrontiers Research Foundation$$c2023
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000258940 520__ $$aThe polyspecific intrathecal immune response (PSIIR), aka MRZ reaction (M = measles, R = rubella, Z = zoster, optionally Herpes simplex virus, HSV) is defined as intrathecal immunoglobulin synthesis (IIS) for two or more unrelated viruses. Although an established cerebrospinal fluid (CSF) biomarker for multiple sclerosis (MS), a chronic autoimmune-inflammatory neurological disease (CAIND) of the central nervous system (CNS) usually starting in young adulthood, the full spectrum of CAINDs with a positive PSIIR remains ill defined.In this retrospective, cross-sectional study, patients with CSF-positive oligoclonal bands (OCB) and - to enrich for non-MS diagnoses - aged ≥50 years were enrolled.Of 415 with PSIIR testing results (MRZ, HSV optional), 76 were PSIIR-positive. Of these, 25 (33%) did not meet the diagnostic criteria for MS spectrum diseases (MS-S) comprising clinically or radiologically isolated syndrome (CIS/RIS) or MS. PSIIR-positive non-MS-S phenotypes were heterogenous with CNS, peripheral nerve and motor neuron involvement and often defied unequivocal diagnostic classification. A rating by neuroimmunology experts suggested non-MS CAINDs in 16/25 (64%). Long-term follow-up available in 13 always showed a chronically progressive course. Four of five responded to immunotherapy. Compared to MS-S patients, non-MS CAIND patients showed less frequent CNS regions with demyelination (25% vs. 75%) and quantitative IgG IIS (31% vs. 81%). MRZ-specific IIS did not differ between both groups, while additional HSV-specific IIS was characteristic for non-MS CAIND patients.In conclusion, PSIIR positivity occurs frequently in non-MS-S patients ≥50 years. Although sometimes apparently coincidental, the PSIIR seems to represent a suitable biomarker for previously unnoticed chronic neurologic autoimmunities, which require further characterization.
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000258940 650_7 $$2Other$$aMRZ reaction
000258940 650_7 $$2Other$$aautoimmue disease
000258940 650_7 $$2Other$$amultiple sclerosis
000258940 650_7 $$2Other$$aneuroinflammation
000258940 650_7 $$2Other$$apolyspecific intrathecal immune response
000258940 7001_ $$aRapp, Daniel$$b1
000258940 7001_ $$aSenel, Makbule$$b2
000258940 7001_ $$0P:(DE-HGF)0$$aHuss, André$$b3
000258940 7001_ $$aDreyhaupt, Jens$$b4
000258940 7001_ $$0P:(DE-2719)9001084$$aKlose, Veronika$$b5
000258940 7001_ $$aSüße, Marie$$b6
000258940 7001_ $$aStürner, Klarissa Hanja$$b7
000258940 7001_ $$aLeypoldt, Frank$$b8
000258940 7001_ $$0P:(DE-2719)9002007$$aTumani, Hayrettin$$b9$$udzne
000258940 7001_ $$aLewerenz, Jan$$b10
000258940 773__ $$0PERI:(DE-600)2564214-5$$a10.3389/fneur.2023.1193015$$gVol. 14, p. 1193015$$p1193015$$tFrontiers in neurology$$v14$$x1664-2295$$y2023
000258940 8564_ $$uhttps://www.frontiersin.org/articles/10.3389/fneur.2023.1193015/full
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