eprintid: 10099251 rev_number: 16 eprint_status: archive userid: 608 dir: disk0/10/09/92/51 datestamp: 2020-06-02 09:32:30 lastmod: 2021-11-02 23:28:55 status_changed: 2020-06-02 09:32:30 type: article metadata_visibility: show creators_name: Chelban, V creators_name: Catereniuc, D creators_name: Aftene, D creators_name: Gasnas, A creators_name: Vichayanrat, E creators_name: Iodice, V creators_name: Groppa, S creators_name: Houlden, H title: An update on MSA: premotor and non-motor features open a window of opportunities for early diagnosis and intervention ispublished: inpress subjects: UCH divisions: UCL divisions: B02 divisions: C07 divisions: D07 divisions: F85 keywords: MSA, Multiple system atrophy, Non-motor features, Premotor phase note: © The Author(s) 2020. This article is licensed under a Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/). abstract: In this review, we describe the wide clinical spectrum of features that can be seen in multiple system atrophy (MSA) with a focus on the premotor phase and the non-motor symptoms providing an up-to-date overview of the current understanding in this fast-growing field. First, we highlight the non-motor features at disease onset when MSA can be indistinguishable from pure autonomic failure or other chronic neurodegenerative conditions. We describe the progression of clinical features to aid the diagnosis of MSA early in the disease course. We go on to describe the levels of diagnostic certainty and we discuss MSA subtypes that do not fit into the current diagnostic criteria, highlighting the complexity of the disease as well as the need for revised diagnostic tools. Second, we describe the pathology, clinical description, and investigations of cardiovascular autonomic failure, urogenital and sexual dysfunction, orthostatic hypotension, and respiratory and REM-sleep behavior disorders, which may precede the motor presentation by months or years. Their presence at presentation, even in the absence of ataxia and parkinsonism, should be regarded as highly suggestive of the premotor phase of MSA. Finally, we discuss how the recognition of the broader spectrum of clinical features of MSA and especially the non-motor features at disease onset represent a window of opportunity for disease-modifying interventions. date: 2020-05-20 date_type: published official_url: https://doi.org/10.1007/s00415-020-09881-6 oa_status: green full_text_type: pub language: eng primo: open primo_central: open_green verified: verified_manual elements_id: 1786825 doi: 10.1007/s00415-020-09881-6 pii: 10.1007/s00415-020-09881-6 lyricists_name: Chelban, Viorica lyricists_name: Houlden, Henry lyricists_id: VCHEL09 lyricists_id: HJHOU44 actors_name: Kalinowski, Damian actors_id: DKALI47 actors_role: owner full_text_status: public publication: Journal of Neurology event_location: Germany citation: Chelban, V; Catereniuc, D; Aftene, D; Gasnas, A; Vichayanrat, E; Iodice, V; Groppa, S; Chelban, V; Catereniuc, D; Aftene, D; Gasnas, A; Vichayanrat, E; Iodice, V; Groppa, S; Houlden, H; - view fewer <#> (2020) An update on MSA: premotor and non-motor features open a window of opportunities for early diagnosis and intervention. Journal of Neurology 10.1007/s00415-020-09881-6 <https://doi.org/10.1007/s00415-020-09881-6>. (In press). Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10099251/1/Chelban_An%20update%20on%20MSA.%20Premotor%20and%20non-motor%20features%20open%20a%20window%20of%20opportunities%20for%20early%20diagnosis%20and%20intervention_AOP.pdf