Skip to main content

Advertisement

SpringerLink
Log in

Significance of the hot-cross bun sign on T2*-weighted MRI for the diagnosis of multiple system atrophy

  • Original Communication
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

Although the sensitive detection of putaminal iron deposition by T2*-weighted imaging (T2*-WI) is of diagnostic value for multiple system atrophy (MSA), the diagnostic significance of the pontine hot-cross bun (HCB) sign with increased ferritin-bound iron in the background remains unknown. We retrospectively evaluated the cases of 33 patients with cerebellar-form MSA (MSA-C) and 21 with MSA of the parkinsonian form (MSA-P) who underwent an MRI study with a 1.5-T system. Visualization of the HCB sign, posterior putaminal hypointensity and putaminal hyperintense rim on T2*-WI was assessed by two neurologists independently using an established visual grade, and were compared with those on T2-weighted imaging (T2-WI). The visual grade of pontine and putaminal signal changes was separately assessed for probable MSA (advanced stage) and possible MSA (early stage). T2*-WI demonstrated significantly higher grades of HCB sign than T2-WI (probable MSA-C, n = 27, p < 0.001; possible MSA-C, n = 6, p < 0.05; probable MSA-P, n = 13, p < 0.01). The visual grade of the HCB sign on T2*-WI in the possible MSA-C patients was comparable to that in the probable MSA-C patients. Although the HCB sign in MSA-P was of lower visual grade than in MSA-C even on T2*-WI, some patients showed evolution of the HCB sign preceding the appearance of the putaminal changes. These findings suggest that T2*-WI is of extreme value for detecting the HCB sign, which is often cited as a hallmark of MSA. The appearance of the HCB sign on T2*-WI might not only support but also improve the diagnosis of MSA.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ et al (2008) Second consensus statement on the diagnosis of multiple system atrophy. Neurology 71:670–676

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  2. Chandran V, Stoessl AJ (2014) Imaging in multiple system atrophy. Neurol Clin Neurosci 2:178–187

    Article  Google Scholar 

  3. Brooks DJ, Seppi K (2009) Proposed neuroimaging criteria for the diagnosis of multiple system atrophy. Mov Disord 24:949–964

    Article  PubMed  Google Scholar 

  4. Kraft E, Trenkwalder C, Auer DP (2002) T2*-weighted MRI differentiates multiple system atrophy from Parkinson’s disease. Neurology 59:1265–1267

    Article  PubMed  Google Scholar 

  5. von Lewinski F, Werner C, Jorn T, Mohr A, Sixel-Doring F, Trenkwalder C (2007) T2*-weighted MRI in diagnosis of multiple system atrophy. A practical approach for clinicians. J Neurol 254:1184–1188

    Article  Google Scholar 

  6. Arabia G, Morelli M, Paglionico S, Novellino F, Salsone M, Giofre L et al (2010) An magnetic resonance imaging T2*-weighted sequence at short echo time to detect putaminal hypointensity in Parkinsonisms. Mov Disord 25:2728–2734

    Article  PubMed  Google Scholar 

  7. Ozawa T, Paviour D, Quinn NP, Josephs KA, Sangha H, Kilford L et al (2004) The spectrum of pathological involvement of the striatonigral and olivopontocerebellar systems in multiple system atrophy: clinicopathological correlations. Brain 127:2657–2671

    Article  PubMed  Google Scholar 

  8. Visanji NP, Collingwood JF, Finnegan ME, Tandon A, House E, Hazrati LN (2013) Iron deficiency in parkinsonism: region-specific iron dysregulation in Parkinson’s disease and multiple system atrophy. J Parkinsons Dis 3:523–537

    CAS  PubMed  Google Scholar 

  9. Horimoto Y, Aiba I, Yasuda T, Ohkawa Y, Katayama T, Yokokawa Y et al (2002) Longitudinal MRI study of multiple system atrophy - when do the findings appear, and what is the course? J Neurol 249:847–854

    Article  PubMed  Google Scholar 

  10. Yabe I, Soma H, Takei A, Fujiki N, Yanagihara T, Sasaki H (2006) MSA-C is the predominant clinical phenotype of MSA in Japan: analysis of 142 patients with probable MSA. J Neurol Sci 249:115–121

    Article  PubMed  Google Scholar 

  11. Geser F, Seppi K, Stampfer-Kountchev M, Kollensperger M, Diem A, Ndayisaba JP et al (2005) The European Multiple System Atrophy-Study Group (EMSA-SG). J Neural Transm 112:1677–1686

    Article  CAS  PubMed  Google Scholar 

  12. Gilman S, May SJ, Shults CW, Tanner CM, Kukull W, Lee VM et al (2005) The North American Multiple System Atrophy Study Group. J Neural Transm 112:1687–1694

    Article  CAS  PubMed  Google Scholar 

  13. Lin DJ, Hermann KL, Schmahmann JD (2014) Multiple system atrophy of the cerebellar type: clinical state of the art. Mov Disord 29:294–304

    Article  PubMed  Google Scholar 

  14. Wenning GK, Tison F, Seppi K, Sampaio C, Diem A, Yekhlef F et al (2004) Development and validation of the Unified Multiple System Atrophy Rating Scale (UMSARS). Mov Disord 19:1391–1402

    Article  PubMed  Google Scholar 

  15. Ito S, Shirai W, Hattori T (2007) Evaluating posterolateral linearization of the putaminal margin with magnetic resonance imaging to diagnose the Parkinson variant of multiple system atrophy. Mov Disord 22:578–581

    Article  PubMed  Google Scholar 

  16. Tha KK, Terae S, Tsukahara A, Soma H, Morita R, Yabe I et al (2012) Hyperintense putaminal rim at 1.5 T: prevalence in normal subjects and distinguishing features from multiple system atrophy. BMC Neurol 12:39. doi:10.1186/1471-2377-12-39

    Article  PubMed Central  PubMed  Google Scholar 

  17. Deguchi K, Sasaki I, Touge T, Tsukaguchi M, Takeuchi H, Kuriyama S (2004) Abnormal baroreceptor-mediated vasopressin release as possible marker in early diagnosis of multiple system atrophy. J Neurol Neurosurg Psychiatry 75:110–115

    PubMed Central  CAS  PubMed  Google Scholar 

  18. Takao M, Kadowaki T, Tomita Y, Yoshida Y, Mihara B (2007) ‘Hot-cross bun sign’ of multiple system atrophy. Intern Med 46:1883

    Article  PubMed  Google Scholar 

  19. Fujii S, Matsusue E, Kinoshita T, Sugihara S, Ohama E, Ogawa T (2007) Hyperintense putaminal rim at 3T reflects fewer ferritin deposits in the lateral marginal area of the putamen. AJNR 28:777–781

    CAS  PubMed  Google Scholar 

  20. Ishigame K, Yagishita A (2010) Diagnostic imaging of multiple system atrophy. Neurol Med (Tokyo, Japan) 73:340–345 (in Japanese)

    Google Scholar 

  21. Watanabe H, Ito M, Fukatsu H, Senda J, Atsuta N, Kaga T et al (2010) Putaminal magnetic resonance imaging features at various magnetic field strengths in multiple system atrophy. Mov Disord 25:1916–1923

    Article  PubMed  Google Scholar 

  22. Kanazawa M, Shimohata T, Toyoshima Y, Tada M, Kakita A, Morita T et al (2009) Cerebellar involvement in progressive supranuclear palsy: a clinicopathological study. Mov Disord 24:1312–1318

    Article  PubMed  Google Scholar 

  23. Lee YC, Liu CS, Wu HM, Wang PS, Chang MH, Soong BW (2009) The ‘hot cross bun’ sign in the patients with spinocerebellar ataxia. Eur J Neurol 16:513–516

    Article  PubMed  Google Scholar 

  24. Muqit MM, Mort D, Miskiel KA, Shakir RA (2001) “Hot cross bun” sign in a patient with parkinsonism secondary to presumed vasculitis. J Neurol Neurosurg Psychiatry 71:565–566

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  25. Soares-Fernandes JP, Ribeiro M, Machado A (2009) “Hot cross bun” sign in variant Creutzfeldt-Jakob disease. AJNR 30:E37

    Article  CAS  PubMed  Google Scholar 

  26. Oji Y, Hatano T, S-i Kubo, Mori A, Nakazato T, Hattori N (2013) Cerebellar ataxia and the “hot cross bun” sign in association with human immunodeficiency virus infection. Neurol Clin Neurosci 1:127

    Article  Google Scholar 

Download references

Acknowledgments

This work was partly supported by JSPS KAKENHI Grant No. 24591297.

Conflicts of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

Ethical standard

The study was approved by the appropriate ethics committee and has therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.

Author information

Authors and Affiliations

  1. Department of Gastroenterology and Neurology, Kagawa University Faculty of Medicine, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa, 761-0793, Japan

    Kazushi Deguchi & Tsutomu Masaki

  2. Department of Neurology, Kagawa University Hospital, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa, 761-0793, Japan

    Kazushi Deguchi, Kodai Kume, Tadayuki Takata & Yohei Kokudo

  3. Department of Intractable Neurological Research, Kagawa University Faculty of Medicine, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa, 761-0793, Japan

    Kazuyo Ikeda & Masaki Kamada

  4. Department of Health Sciences, Kagawa University Faculty of Medicine, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa, 761-0793, Japan

    Tetsuo Touge

  5. Department of Radiology, Osaka Neurosurgical Hospital, 378-1 Sanmyo-cho, Takamatsu, Kagawa, 761-8083, Japan

    Naomi Honjo

Authors
  1. Kazushi Deguchi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kazuyo Ikeda
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Kodai Kume
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Tadayuki Takata
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Yohei Kokudo
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Masaki Kamada
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Tetsuo Touge
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Naomi Honjo
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Tsutomu Masaki
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Kazushi Deguchi.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Deguchi, K., Ikeda, K., Kume, K. et al. Significance of the hot-cross bun sign on T2*-weighted MRI for the diagnosis of multiple system atrophy. J Neurol 262, 1433–1439 (2015). https://doi.org/10.1007/s00415-015-7728-1

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-015-7728-1

Keywords

Search

Navigation