| Title: |
Inaugural dropped head syndrome and camptocormia in inflammatory myopathies: a retrospective study |
| Authors: |
Robert, Marie; Lessard, Lola, E R; Bouhour, Françoise; Petiot, Philippe; Fenouil, Tanguy; Svahn, Juliette; Fiscus, Julie; Fabien, Nicole; Perard, Laurent; Robinson, Philip; Durieu, Isabelle; Coury, Fabienne; Streichenberger, Nathalie; Hot, Arnaud; Gallay, Laure |
| Contributors: |
Hôpital Edouard Herriot CHU - HCL; Hospices Civils de Lyon (HCL); Service d'Electroneuromyographie et Service de Neurologie C Hôpital Pierre Wertheimer - HCL; Hôpital neurologique et neurochirurgical Pierre Wertheimer CHU - HCL; Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL); Centre de Recherche en Cancérologie de Lyon (UNICANCER/CRCL); Centre Léon Bérard Lyon -Université Claude Bernard Lyon 1 (UCBL); Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS); Centre Hospitalier Lyon Sud CHU - HCL (CHLS); Centre hospitalier Saint Joseph - Saint Luc Lyon; Physiopathologie, diagnostic et traitements des maladies osseuses / Pathophysiology, Diagnosis & Treatments of Bone Diseases (LYOS); Université Claude Bernard Lyon 1 (UCBL); Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM); Physiopathologie et génétique du neurone et du muscle (PGNM); Université de Genève = University of Geneva (UNIGE) |
| Source: |
ISSN: 1462-0324. |
| Publisher Information: |
CCSD; Oxford University Press (OUP) |
| Publication Year: |
2023 |
| Collection: |
HAL Lyon 1 (University Claude Bernard Lyon 1) |
| Subject Terms: |
dropped head syndrome; camptocormia; inflammatory myopathies; myositis; [SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system; [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie |
| Description: |
International audience ; Objectives: Inaugural axial muscle involvement, defined as dropped head syndrome (DHS) and/or camptocormia (CC), is poorly described in inflammatory myopathies (IM). This study aimed to further characterize IM patients with inaugural DHS/CC, their outcome and care management.Methods: This retrospective study included IM patients diagnosed between 2000 and 2021. The main inclusion criterion was IM revealed by axial muscle deficit (DHS/CC).Results: Twenty-seven patients were included; median (IQR) age at first symptoms was 66.0 years (55.5–75.0); 21 were female (77.8%). There were nine IBM, 33.3%, nine overlap myositis (OM, 33.3%), five DM, 18.5%, two immune checkpoint inhibitor-related myositis (7.4%), one focal myositis (3.7%) and one myositis with anti-Hu antibodies (3.7%). Age at first symptoms was ≤70 years in 16 patients (59.3%), including all DM patients and 8/9 OM patients (88.9%). In this group, partial remission of the disease was obtained in 9/16 (56.3%) and complete remission in 1/16 patients (6.3%); regression of DHS/CC was achieved in 3/16 patients (18.8%). Conversely, in the group of 11 patients aged >70 years at first symptoms, there were eight IBM (72.7%). Partial remission was obtained in 5/11 patients (45.5%), the disease was stable in 6/11 patients (54.5%); no complete remission was obtained nor regression of DHS/CC.Conclusion: The analysis of IM patients with inaugural DHS/CC delineates two groups of patients according to the age at first symptoms in terms of clinical and outcome specificities, and proposes an adapted diagnostic and care management approach to prevent long-term complications. |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| DOI: |
10.1093/rheumatology/kead347 |
| Availability: |
https://hal.science/hal-04490558; https://hal.science/hal-04490558v1/document; https://hal.science/hal-04490558v1/file/kead347.pdf; https://doi.org/10.1093/rheumatology/kead347 |
| Rights: |
https://about.hal.science/hal-authorisation-v1/ ; info:eu-repo/semantics/OpenAccess |
| Accession Number: |
edsbas.CED9AC40 |
| Database: |
BASE |