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Original research
Early atypical signs and insula hypometabolism predict survival in multiple system atrophy
  1. Stephan Grimaldi1,
  2. Mohamed Boucekine2,
  3. Tatiana Witjas1,3,
  4. Frederique Fluchere1,
  5. Jean-Philippe Azulay1,
  6. Eric Guedj4,5,6,
  7. Alexandre Eusebio1,3
  1. 1 Department of Neurology and Movement Disorders, University Hospital La Timone, Marseille, France
  2. 2 EA3279 Self-perceived Health Assessment Research Unit, Aix-Marseille University, Marseille, France
  3. 3 UMR 7289, Institut de Neurosciences de la Timone, Marseille, France
  4. 4 Service Central de Biophysique et Médecine Nucléaire, University Hospital La Timone, Marseille, France
  5. 5 CNRS, Ecole Centrale Marseille, UMR 7249, Institut Fresnel, Marseille, France
  6. 6 CERIMED, Aix-Marseille University, Marseille, France
  1. Correspondence to Dr Stephan Grimaldi, Department of Neurology and Movement Disorders, Assistance Publique Hopitaux de Marseille, Marseille, France; stephan.grimaldi{at}gmail.com

Abstract

Objective We aim to search for predictors of survival among clinical and brain 18F-FDG positron emission tomography (PET) metabolic features in our cohort of patients with multiple system atrophy (MSA).

Methods We included patients with a ‘probable’ MSA diagnosis for whom a clinical evaluation and a brain PET were performed early in the course of the disease (median 3 years, IQR 2–5). A retrospective analysis was conducted using standardised data collection. Brain PET metabolism was characterised using the Automated Anatomical Labelling Atlas. A Cox model was applied to look for factors influencing survival. Kaplan-Meier method estimated the survival rate. We proposed to develop a predictive ‘risk score’, categorised into low-risk and high-risk groups, using significant variables entered in multivariate Cox regression analysis.

Results Eighty-five patients were included. The overall median survival was 8 years (CI 6.64 to 9.36). Poor prognostic factors were orthostatic hypotension (HR=6.04 (CI 1.58 to 23.12), p=0.009), stridor (HR=3.41 (CI 1.31 to 8.87), p=0.012) and glucose PET hypometabolism in the left insula (HR=0.78 (CI 0.66 to 0.92), p=0.004). Good prognostic factors were time to diagnosis (HR=0.68 (CI 0.54 to 0.86), p=0.001) and use of selective serotonin reuptake inhibitor (SSRI) (HR=0.17 (CI 0.06 to 0.46), p<0.001). The risk score revealed a 5-year gap separating the median survival of the two groups obtained (5 years vs 10 years; HR=5.82 (CI 2.94 to 11.49), p<0.001).

Conclusion The clinical prognosis factors we have described support published studies. Here, we also suggest that brain PET is of interest for prognosis assessment and in particular in the search for left insula hypometabolism. Moreover, SSRIs are a potential drug candidate to slow the progression of the disease.

Data availability statement

Data are available upon reasonable request.

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Data availability statement

Data are available upon reasonable request.

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Footnotes

  • Contributors SG: conception and design of the study, acquisition and analysis of data, drafting the manuscript and figures, approval of the final draft. MB: analysis of data, drafting the manuscript and figures, approval of the final draft. TW, FF: acquisition of data, approval of the final draft. J-PA: acquisition of data, drafting the manuscript, approval of the final draft. EG, AE: acquisition and analysis of data, drafting the manuscript, approval of the final draft.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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