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Progressive post infectious neurological syndromes with a poor outcome: Long term follow-up and neurofilament light chain quantification

Businaro, P; Currò, R; Vegezzi, E; Diamanti, L; Bini, P; Cosentino, G; Alfonsi, E; ... Marchioni, E; + view all (2023) Progressive post infectious neurological syndromes with a poor outcome: Long term follow-up and neurofilament light chain quantification. Multiple Sclerosis and Related Disorders , 76 , Article 104781. 10.1016/j.msard.2023.104781. Green open access

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Abstract

Postinfectious neurological syndromes (PINS), among which acute disseminated encephalomyelitis (ADEM), are inflammatory and mostly monophasic disorders. We previously reported that PINS patients can show relapses, or even disease progression. Here we describe a cohort of patients with progressive-PINS and >5 years of follow-up, that developed a progressive worsening without radiological/cerebrospinal fluid analysis evidence of inflammation. At onset 5 patients fulfilled diagnostic criteria for ADEM and none for MS. Progression occurred after a median of 22 months from onset (in 4/7 after 1/more relapses), manifesting as ascending tetraparesis with bulbar functions involvement in 5/7. Five/7 patients received high dose steroids and/or IvIG and 6/7 Rituximab(n = 4) and/or cyclophosphamide(n = 2), with no impact on disease progression in 6/7. NfL levels were higher in patients with progressive-PINS compared to monophasic-ADEM (p = 0.023) and healthy controls (p = 0.004). Progression is rare, but possible, in PINS. Immunotherapy seems to be ineffective in these patients, and elevated serum NfL in serum suggest persistent axonal damage.

Type: Article
Title: Progressive post infectious neurological syndromes with a poor outcome: Long term follow-up and neurofilament light chain quantification
Location: Netherlands
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.msard.2023.104781
Publisher version: https://doi.org/10.1016/j.msard.2023.104781
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher's terms and conditions.
Keywords: Acute disseminated encephalomyelitis, Demyelinating disease, Immunotherapy, Neurofilament light chain, Neuroinflammatory disease, Post infectious neurological syndrome
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases
URI: https://discovery.ucl.ac.uk/id/eprint/10174567
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