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J Neurol Neurosurg Psychiatry. 2021 Apr 23; doi: 10.1136/jnnp-2020-324823. Epub 2021 Apr 23.

Early atypical signs and insula hypometabolism predict survival in multiple system atrophy.

Journal of neurology, neurosurgery, and psychiatry

Stephan Grimaldi, Mohamed Boucekine, Tatiana Witjas, Frederique Fluchere, Jean-Philippe Azulay, Eric Guedj, Alexandre Eusebio

Affiliations

  1. Department of Neurology and Movement Disorders, University Hospital La Timone, Marseille, France [email protected].
  2. EA3279 Self-perceived Health Assessment Research Unit, Aix-Marseille University, Marseille, France.
  3. Department of Neurology and Movement Disorders, University Hospital La Timone, Marseille, France.
  4. UMR 7289, Institut de Neurosciences de la Timone, Marseille, France.
  5. Service Central de Biophysique et Médecine Nucléaire, University Hospital La Timone, Marseille, France.
  6. CNRS, Ecole Centrale Marseille, UMR 7249, Institut Fresnel, Marseille, France.
  7. CERIMED, Aix-Marseille University, Marseille, France.

PMID: 33893230 DOI: 10.1136/jnnp-2020-324823

Abstract

OBJECTIVE: We aim to search for predictors of survival among clinical and brain

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.

© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.

Conflict of interest statement

Competing interests: None declared.

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