Skip to main content

Advertisement

SpringerLink
Log in

Reduced cardiac 123I-metaiodobenzylguanidine uptake in patients with spinocerebellar ataxia type 2: a comparative study with Parkinson’s disease

  • Original Article
  • Published:
European Journal of Nuclear Medicine and Molecular Imaging Aims and scope Submit manuscript

Abstract

Purpose

Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disorder characterized by cerebellar ataxia, supranuclear ophthalmoplegia, and peripheral neuropathy. Autonomic nervous system dysfunction is often present. This study evaluated the cardiac sympathetic function in patients with SCA2 using 123I-metaiodobenzylguanidine (MIBG) in comparison with patients with Parkinson’s disease (PD) and control subjects.

Methods

Nine patients with SCA2, nine patients with PD, and nine control subjects underwent 123I-MIBG imaging studies from which early and late heart-to-mediastinum (H/M) ratios and myocardial washout rates were calculated.

Results

Early (F = 12.3, p < 0.0001) and late (F = 16.8, p < 0.0001) H/M ratios were significantly different among groups. In controls, early and late H/M ratios (2.2 ± 0.12 and 2.1 ± 0.20) were significantly higher than in patients with SCA2 (1.9 ± 0.23 and 1.8 ± 0.20, both p < 0.05) and with patients with PD (1.7 ± 0.29 and 1.4 ± 0.35, both p < 0.001). There was also a significant difference in washout rates among groups (F = 11.7, p < 0.0001). In controls the washout rate (19.9 ± 9.6 %) was significantly lower (p < 0.005) than in patients with PD (51.0 ± 23.7 %), but not different from that in SCA2 patients (19.5 ± 9.4 %). In SCA2 patients, in a multivariable linear regression analysis only the Scale for the Assessment and Rating of Ataxia score was independently associated with early H/M ratio (β = −0.12, p < 0.05).

Conclusion

123I-MIBG myocardial scintigraphy demonstrated an impairment of cardiac sympathetic function in patients with SCA2, which was less marked than in PD patients. These results suggest that 123I-MIBG cardiac imaging could become a useful tool for analysing the pathophysiology of SCA2.

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
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Taki J, Yoshita M, Yamada M, Tonami N. Significance of 123I-MIBG scintigraphy as a pathophysiological indicator in the assessment of Parkinson’s disease and related disorders: it can be a specific marker for Lewy body disease. Ann Nucl Med. 2004;18:453–61.

    Article  PubMed  Google Scholar 

  2. Damier P, Hirsch EC, Agid Y, Graybiel AM. The substantia nigra of the human brain. II. Patterns of loss of dopamine-containing neurons in Parkinson’s disease. Brain. 1999;122:1437–48.

    Article  PubMed  Google Scholar 

  3. Wakabayashi K, Tanji K, Mori F, Takahashi H. The Lewy body in Parkinson’s disease: molecules implicated in the formation and degradation of alpha-synuclein aggregates. Neuropathology. 2007;27:494–506.

    Article  PubMed  Google Scholar 

  4. Wolters EC. Non-motor extranigral signs and symptoms in Parkinson’s disease. Parkinsonism Relat Disord. 2009;15:S6–12.

    Article  PubMed  Google Scholar 

  5. Oka H, Toyoda C, Yogo M, Mochio S. Reduced cardiac 123I-MIBG uptake reflects cardiac sympathetic dysfunction in de novo Parkinson’s disease. J Neural Transm. 2011;118:1323–7.

    Article  PubMed  Google Scholar 

  6. Treglia G, Cason E, Stefanelli A, Cocciolillo F, Di Giuda D, Fagioli G, et al. MIBG scintigraphy in differential diagnosis of Parkinsonism: a meta-analysis. Clin Auton Res. 2012;22:43–55.

    Article  PubMed  Google Scholar 

  7. Magaña JJ, Velázquez-Pérez L, Cisneros B. Spinocerebellar ataxia type 2: clinical presentation, molecular mechanisms, and therapeutic perspectives. Mol Neurobiol. 2013;47:90–104.

    Article  PubMed  Google Scholar 

  8. Durr A. Autosomal dominant cerebellar ataxias: polyglutamine expansions and beyond. Lancet Neurol. 2010;9:885–94.

    Article  PubMed  CAS  Google Scholar 

  9. De Joanna G, De Rosa A, Salvatore E, Castaldo I, De Luca N, Izzo R, et al. Autonomic nervous system abnormalities in spinocerebellar ataxia. J Neurol Sci. 2008;275:60–3.

    Article  PubMed  Google Scholar 

  10. Yomono HS, Kurisaki H, Hebisawa A, Sakiyama Y, Saito Y, Murayama S. Autopsy case of SCA2 with Parkinsonian phenotype. Rinsho Shinkeigaku. 2010;50:156–62.

    Article  PubMed  Google Scholar 

  11. Rascol O, Schelosky L. 123I-metaiodobenzylguanidine scintigraphy in Parkinson’s disease and related disorders. Mov Disord. 2009;24:S732–41.

    Article  PubMed  Google Scholar 

  12. Schmitz-Hübsch T, du Montcel ST, Baliko L, Berciano J, Boesch S, Depondt C, et al. Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology. 2006;66:1717–20.

    Article  PubMed  Google Scholar 

  13. Yeh TH, Lu CS, Chou YH, Chong CC, Wu T, Han NH, et al. Autonomic dysfunction in Machado-Joseph disease. Arch Neurol. 2005;62:630–6.

    Article  PubMed  Google Scholar 

  14. Fahn S, Elton RL; Members of the UPDRS Development Committee. The Unified Parkinson’s disease rating scale. In: Fahn S, Marsden CD, Calne DB, Goldstein M, editors. Recent developments in Parkinson’s disease, Vol. 2. Florham Park: Macmillan Healthcare Information; 1987. p. 153–63. 293–304.

    Google Scholar 

  15. Hoehn MM, Yahr MD. Parkinsonism: onset, progression and mortality. Neurology. 1967;17:427–42.

    Article  PubMed  CAS  Google Scholar 

  16. Flotats A, Carrió I, Agostini D, Le Guludec D, Marcassa C, Schäfers M, et al.; EANM Cardiovascular Committee; European Council of Nuclear Cardiology. Proposal for standardization of 123I-metaiodobenzylguanidine (MIBG) cardiac sympathetic imaging by the EANM Cardiovascular Committee and the European Council of Nuclear Cardiology. Eur J Nucl Med Mol Imaging. 2010;37:1802–12.

    Article  PubMed  Google Scholar 

  17. Dürr A, Smadja D, Cancel G, Lezin A, Stevanin G, Mikol J, et al. Autosomal dominant cerebellar ataxia type I in Martinique (French West Indies). Clinical and neuropathological analysis of 53 patients from three unrelated SCA2 families. Brain. 1995;118:157–81.

    Article  Google Scholar 

  18. Gierga K, Bürk K, Bauer M, Orozco Diaz G, Auburger G, Schultz C, et al. Involvement of the cranial nerves and their nuclei in spinocerebellar ataxia type 2 (SCA2). Acta Neuropathol. 2005;109:617–31.

    Article  PubMed  CAS  Google Scholar 

  19. Kazuta T, Hayashi M, Shimizu T, Iwasaki A, Nakamura S, Hirai S. Autonomic dysfunction in Machado-Joseph disease assessed by iodine123-labeled metaiodobenzylguanidine myocardial scintigraphy. Clin Auton Res. 2000;10:111–5.

    Article  PubMed  CAS  Google Scholar 

  20. Pellegrino T, Petretta M, De Luca S, Paolillo S, Boemio A, Carotenuto R, et al. Observer reproducibility of results from a low-dose (123)I-metaiodobenzylguanidine cardiac imaging protocol in patients with heart failure. Eur J Nucl Med Mol Imaging. 2013. doi:10.1007/s00259-013-2461-4.

  21. Kline RC, Swanson DP, Wieland DM, Thrall JH, Gross MD, Pitt B, et al. Myocardial imaging in man with I-123 meta-iodobenzylguanidine. J Nucl Med. 1981;22:129–32.

    PubMed  CAS  Google Scholar 

  22. Sisson JC, Shapiro B, Meyers L, Mallette S, Mangner TJ, Wieland DM, et al. Metaiodobenzylguanidine to map scintigraphically the adrenergic nervous system in man. J Nucl Med. 1987;28:1625–36.

    PubMed  CAS  Google Scholar 

  23. Estorch M, Carrió I, Berná L, López-Pousa J, Torres G. Myocardial iodine-labeled metaiodobenzylguanidine 123 uptake relates to age. J Nucl Cardiol. 1995;2:126–32.

    PubMed  CAS  Google Scholar 

  24. Ji SY, Travin MI. Radionuclide imaging of cardiac autonomic innervation. J Nucl Cardiol. 2010;17:655–66.

    Article  PubMed  Google Scholar 

  25. Netravathi M, Sathyaprabha TN, Jayalaxmi K, Datta P, Nirmala M, Pal PK. A comparative study of cardiac dysautonomia in autosomal dominant spinocerebellar ataxias and idiopathic sporadic ataxias. Acta Neurol Scand. 2009;120:204–9.

    Article  PubMed  CAS  Google Scholar 

  26. Takao M, Aoyama M, Ishikawa K, Sakiyama Y, Yomono H, Saito Y, et al. Spinocerebellar ataxia type 2 is associated with Parkinsonism and Lewy body pathology. BMJ Case Rep. 2011;2011:bcr0120113685.

    Article  PubMed  Google Scholar 

  27. Varrone A, Salvatore E, De Michele G, Barone P, Sansone V, Pellecchia MT, et al. Reduced striatal [123I]FP-CIT binding in SCA2 patients without parkinsonism. Ann Neurol. 2004;553:426–30.

    Article  Google Scholar 

  28. Estrada R, Galarraga J, Orozco G, Nodarse A, Auburger G. Spinocerebellar ataxia 2 (SCA2): morphometric analyses in 11 autopsies. Acta Neuropathol. 1999;97:306–10.

    Article  PubMed  CAS  Google Scholar 

  29. Filla A, De Michele G, Banfi S, Santoro L, Perretti A, Cavalcanti F, et al. Has spinocerebellar ataxia type 2 a distinct phenotype? Genetic and clinical study of an Italian family. Neurology. 1995;45:793–6.

    Article  PubMed  CAS  Google Scholar 

  30. van de Warrenburg BP, Notermans NC, Schelhaas HJ, van Alfen N, Sinke RJ, Knoers NV, et al. Peripheral nerve involvement in spinocerebellar ataxias. Arch Neurol. 2004;61:257–61.

    Article  PubMed  Google Scholar 

  31. Perretti A, Santoro L, Lanzillo B, Filla A, De Michele G, Barbieri F, et al. Autosomal dominant cerebellar ataxia type I: multimodal electrophysiological study and comparison between SCA1 and SCA2 patients. J Neurol Sci. 1996;142:45–53.

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

This study was supported in part by the Italian Ministry of Research (MIUR)-MEdical Research in ITaly (MERIT) project no. RBNE08LN4P and by a grant from the Italian Ministry of Education, University, and Research (PRIN 2010-2011 20108WT59Y-007)

Conflicts of interest

None.

Author information

Authors and Affiliations

  1. Department of Neurosciences and Reproductive and Odontostomatologic Sciences, University Federico II, Naples, Italy

    Anna De Rosa, Maria Fulvia De Leva, Gennaro Maddaluno, Alessandro Filla & Giuseppe De Michele

  2. Institute of Biostructure and Bioimaging, National Council of Research, Naples, Italy

    Sabina Pappatà & Teresa Pellegrino

  3. Department of Translational Medical Sciences, University Federico II, Naples, Italy

    Mario Petretta

  4. SDN Foundation, Institute of Diagnostic and Nuclear Development, Naples, Italy

    Giovanni Fiumara

  5. Department of Advanced Biomedical Sciences, University Federico II, Naples, Italy

    Raffaella Carotenuto & Alberto Cuocolo

Authors
  1. Anna De Rosa
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sabina Pappatà
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Teresa Pellegrino
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Maria Fulvia De Leva
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Gennaro Maddaluno
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Giovanni Fiumara
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Raffaella Carotenuto
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Mario Petretta
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Alessandro Filla
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Giuseppe De Michele
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Alberto Cuocolo
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Alberto Cuocolo.

Rights and permissions

Reprints and permissions

About this article

Cite this article

De Rosa, A., Pappatà, S., Pellegrino, T. et al. Reduced cardiac 123I-metaiodobenzylguanidine uptake in patients with spinocerebellar ataxia type 2: a comparative study with Parkinson’s disease. Eur J Nucl Med Mol Imaging 40, 1914–1921 (2013). https://doi.org/10.1007/s00259-013-2524-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00259-013-2524-6

Keywords

Search

Navigation