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. 2020 Nov 27;20(1):432.
doi: 10.1186/s12883-020-02007-5.

A distinct neuromelanin magnetic resonance imaging pattern in parkinsonian multiple system atrophy

Rita Moiron Simões  1   2 , Ana Castro Caldas  2   3 , Joana Grilo  3   4   5 , Daisy Correia  3   4 , Carla Guerreiro  3   6   7 , Patrícia Pita Lobo  2   3   8 , Anabela Valadas  2   3   8 , Marguerita Fabbri  3   9 , Leonor Correia Guedes  2   3   8 , Miguel Coelho  3   8 , Mario Miguel Rosa  4   8 , Joaquim J Ferreira  10   11   12 , Sofia Reimão  4   6   7
Affiliations

A distinct neuromelanin magnetic resonance imaging pattern in parkinsonian multiple system atrophy

Rita Moiron Simões et al. BMC Neurol. .

Abstract

Background: Parkinsonian variant of multiple system atrophy is a neurodegenerative disorder frequently misdiagnosed as Parkinson's disease. No early imaging biomarkers currently differentiate these disorders.

Methods: Simple visual imaging analysis of the substantia nigra and locus coeruleus in neuromelanin-sensitive magnetic resonance imaging and nigrosome 1 in susceptibility-weighted sequences was performed in thirty patients with parkinsonian variant of multiple system atrophy fulfilling possible/probable second consensus diagnostic criteria. The neuromelanin visual pattern was compared to patients with Parkinson's disease with the same disease duration (n = 10) and healthy controls (n = 10). Substantia nigra semi-automated neuromelanin area/signal intensity was compared to the visual data.

Results: Groups were similar in age, sex, disease duration, and levodopa equivalent dose. Hoehn & Yahr stage was higher in parkinsonian multiple system atrophy patients, 69% of whom had normal neuromelanin size/signal, significantly different from Parkinson's disease patients, and similar to controls. Nigrosome 1 signal was lost in 74% of parkinsonian multiple system atrophy patients. Semi-automated neuromelanin substantia nigra signal, but not area, measurements were able to differentiate groups.

Conclusions: In patients with parkinsonism, simple visual magnetic resonance imaging analysis showing normal neuromelanin substantia nigra and locus coeruleus, combined with nigrosome 1 loss, allowed the distinction of the parkinsonian variant of multiple system atrophy from Parkinson's disease and healthy controls. This easy and widely available method was superior to semi-automated measurements in identifying specific imaging changes in substantia nigra and locus coeruleus.

Keywords: MRI; Multiple system atrophy; Neuromelanin; Nigrosome 1; Susceptibility-weighted imaging.

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Conflict of interest statement

RMS has no conflict of interest relevant to this work but is currently a consultant for BIAL – PORTELA & C.a, S.A. and has been a consultant for Zambon and AbbVie.

JJF has no conflict of interest relevant to this work but reports having received grants from GlaxoSmithKline, Grunenthal, Fundação MSD (Portugal), TEVA, MSD, Allergan, Novartis, Medtronic. He received consultancy fees from GlaxoSmithKline, Novartis, TEVA, Lundbeck, Solvay, BIAL, Merck-Serono, Merz, Ipsen, Biogen, Acadia, Allergan, Abbvie, Sunovion Pharmaceuticals. He is employed by Faculdade de Medicina de Lisboa and CNS - Campus Neurológico Sénior. He also participated in advisory boards for Bial and expert testimony to Novartis.

MF has no conflict of interest relevant to this work but received honoraria to speak from AbbVie.

Remaining authors have no conflict of interest nor relevant disclosures for the past 12 months.

Figures

Fig. 1
Fig. 1
Imaging analysis and available sample size for each group and MRI modality
Fig. 2
Fig. 2
Visual analysis of SN and LC in NM-MRI and nigrosome-1 in SWI in patients with MSA-P
Fig. 3
Fig. 3
Neuromelanin-sensitive MRI axial images of SN (a-c) and LC (d-f) in a patient with MSA-P (a, d), PD with 2–5 years of duration (b, e) and a healthy control (c, f)
Fig. 4
Fig. 4
Comparison of the visual pattern of SN size, SN signal intensity and LC signal intensity in NM-MRI, in MSA-P, PD 2–5 years of duration and healthy controls
Fig. 5
Fig. 5
Classification of NM-MRI visual SN pattern in each group according to the radiological diagnosis of PD (probable PD, possible PD, not suggestive of PD and uncertain)
Fig. 6
Fig. 6
Ability of quantitative methods to discriminate between MSA-P, PD2_5y and HC, evaluated by ROC curves
See this image and copyright information in PMC

References

    1. Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ, et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008;71:670–676. doi: 10.1212/01.wnl.0000324625.00404.15. - DOI - PMC - PubMed
    1. Wenning GK, Ben-Shlomo Y, Magalhaes M, Daniel SE, Quinn NP. Clinicopathological study of 35 cases of multiple system atrophy. J Neurol Neurosurg Psychiatry. 1995;58(2):160–166. doi: 10.1136/jnnp.58.2.160. - DOI - PMC - PubMed
    1. Hughes A, Daniel S, Ben Shlomo Y, Lees AJ. The accuracy of diagnosis of Parkinsonian syndromes in a specialist movement disorder service. Brain. 2002;125(4):861. doi: 10.1093/brain/awf080. - DOI - PubMed
    1. Hughes A, Daniel S, Kilford L, Lees A. The accuracy of clinical diagnosis of idiopathic Parkinson’s disease: a clinicopathological study of 100 cases. J Neurol Neurosurg Psychiatry. 1992;55:181–184. doi: 10.1136/jnnp.55.3.181. - DOI - PMC - PubMed
    1. Wenning GK, Tison F, Ben Shlomo Y, Daniel SE, Quinn NP. Multiple system atrophy: a review of 203 pathologically proven cases. Mov Disord. 1997;12(2):133–147. doi: 10.1002/mds.870120203. - DOI - PubMed

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