Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 May;267(5):1269-1277.
doi: 10.1007/s00415-020-09702-w. Epub 2020 Jan 14.

Brain MRI of multiple system atrophy of cerebellar type: a prospective study with implications for diagnosis criteria

G Carré  1   2 , J L Dietemann  3 , O Gebus  1 , S Montaut  1 , O Lagha-Boukbiza  1 , T Wirth  1 , S Kremer  3 , I J Namer  4 , M Anheim  1   5   6 , C Tranchant  7   8   9
Affiliations

Brain MRI of multiple system atrophy of cerebellar type: a prospective study with implications for diagnosis criteria

G Carré et al. J Neurol. 2020 May.

Abstract

Aim: The second consensus statement for the diagnosis of multiple system atrophy type cerebellar (MSA-C) includes pons and middle cerebellar peduncle (MCP) atrophy as MRI features. However, other MRI abnormalities such as MCP hyperintensity, hot cross bun sign (HCB), putaminal hypointensity and hyperintense putaminal rim have been described.

Objectives: To evaluate, in patients with sporadic late-onset cerebellar ataxia (SLOCA), the discriminative value of several MRI features for the diagnosis of MSA-C, to follow their evolution during the course of MSA-C, and to search for correlations between these MRI features and clinical signs.

Methods: Consecutive patients referred for SLOCA underwent comprehensive clinical evaluation and laboratory investigations, brain MRI, DaTscan and a 1-year follow-up.

Results: Among 80 patients, 26 had MSA-C, 22 another diagnosis, and 32 no diagnosis at the end of the follow-up. At baseline, MCP hyperintensity and HCB were more frequent in patients finally diagnosed with MSA-C than in other patients with SLOCA (p < 0.0001), and had the highest specificity (98.5%) and positive predictive value (91.7%) for the diagnosis of MSA-C, compared to all other MRI signs. The most relevant MRI sequence regarding HCB sign was the T2-proton density (DP) weighted. All MRI features were more frequent with disease duration. No correlation was found between any MRI feature and neither clinical data, nor dopaminergic neuronal loss (p = 0.5008), except between vermis atrophy and UPDRSIII score.

Conclusion: MCP hyperintensity and HCB sign should be added into the list of additional features of possible MSA-C. MRI signal abnormalities suggestive of MSA-C should be searched for in suitable sequence.

Keywords: DaTscan; Hot cross bun sign; Late-onset cerebellar ataxia; Magnetic resonance imaging; Multiple system atrophy.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Gebus O, Montaut S, Monga B, Wirth T, Cheraud C, Rego AC, Zinchenko I, Carré G, Hamdaoui M, Hautecloque G, Nguyen-Them L, Lannes B, Chanson JB, Lagha-Boukbiza O, Fleury MC, Devys D, Nicolas G, Rudolf G, Bereau M, Mallaret M, Renaud M, Acquaviva M, Koenig M, Koob M, Kremer S, Namer IJ, Cazeneuve C, -Laguna A, Tranchant C, Anheim M (2017) Deciphering the causes of sporadic late-onset cerebellar ataxias: a prospective study with implications for diagnostic work. J Neurol 264(6):1118–1126 - PubMed
    1. Hadjivassiliou M, Martindale J, Shanmugarajah P, Grünewald RA, Sarrigiannis PG, Beauchamp N, Garrard K, Warburton R, Sanders DS, Friend D, Duty S, Taylor J, Hoggard N (2017) Causes of progressive cerebellar ataxia: prospective evaluation of 1500 patients. J Neurol Neurosurg Psychiatry 88(4):301–309 - PubMed
    1. Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ, Wood NW, Colosimo C, Dürr A, Fowler CJ, Kaufmann H, Klockgether T, Lees A, Poewe W, Quinn N, Revesz T, Robertson D, Sandroni P, Seppi K, Vidailhet M (2008) Second consensus statement on the diagnosis of multiple system atrophy. Neurology 71(9):670–676 - PubMed - PMC
    1. Lewinski F, Werner C, Jörn T, Mohr A, Sixel-Döring F, Trenkwalder C (2007) T2*-weighted MRI in diagnosis of multiple system atrophy: a practical approach for clinicians. J Neurol 254(9):1184–1188
    1. Righini A, Antonini A, Ferrarini M, de Notaris R, Canesi M, Triulzi F, Pezzoli G (2002) Thin section MR study of the basal ganglia in the differential diagnosis between striatonigral degeneration and Parkinson disease. J Comput Assist Tomogr 26(2):266–271 - PubMed

MeSH terms

LinkOut - more resources