Skip to main content

Advertisement

SpringerLink
Log in

3-T MR neurography of lumbo-sacral plexus in hereditary transthyretin-related amyloidosis with polyneuropathy

  • Magnetic Resonance
  • Published:
European Radiology Aims and scope Submit manuscript

Abstract

Objectives

Our aim was to evaluate the ability of magnetic resonance neurography (MRN) of the lumbo-sacral plexus (LSP) to distinguish patients with hereditary transthyretin-related amyloidosis with polyneuropathy (ATTRv-PN) from asymptomatic variant carriers (AVC) and healthy controls and to assess its prognostic value.

Methods

Three-Tesla MRN was performed in 25 consecutive ATTRv-PN patients, 18 AVC, and 10 controls including T2-w DIXON and DWI MR sequences. Two blinded readers independently assessed LSP root diameter and intraneural signal on the MRN images of each subject. MRN findings were compared between groups and correlated with clinical impairment scored on the Neuropathy Impairment Score (NIS) and the modified Polyneuropathy Disability score (mPND).

Results

The agreement between readers on MRN images was excellent (Cohen’s kappa = 0.82). LSP root enlargement was significantly more frequent in ATTRv-PN patients compared to AVC (ratio = 4.38, p = 0.038). Increased LSP root intraneural signal on T2-w images was significantly more frequent in ATTRv-PN patients compared to AVC (ratio = 3.4, p = 0.016). In contrast, there were no MRN abnormalities in controls. In ATTRv-PN patients, LSP root enlargement was associated with higher mPND scores (p = 0.03) and increased intraneural signal on T2-w images was associated with significantly higher NIS and mPND scores (p = 0.004 and 0.02, respectively).

Conclusions

MRN of the LSP can help differentiate ATTRv-PN patients from AVC. LSP root enlargement and increased intraneural signal are significantly associated with clinical impairment, suggesting potential implications for patient care.

Key Points

• ATTRv-PN patients showed abnormal LSP changes on MRN.

• MRN of the LSP can help to differentiate ATTRv-PN patients from AVC and healthy controls.

• LSP root enlargement and increased intraneural signal were significantly associated with clinical impairment in ATTRv-PN patients.

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

Abbreviations

ATTRv-PN:

Hereditary transthyretin-related amyloidosis with polyneuropathy

AVC:

Asymptomatic variant carriers

LSP:

Lumbo-sacral plexus

mPND:

Modified Polyneuropathy Disability Score

MRN:

Magnetic resonance neurography

NIS:

Neuropathy Impairment Score

TTR:

Transthyretin

References

  1. Plante-Bordeneuve V (2018) Transthyretin familial amyloid polyneuropathy: an update. J Neurol 265:976–983

    Article  CAS  Google Scholar 

  2. Gillmore JD, Maurer MS, Falk RH et al (2016) Nonbiopsy diagnosis of cardiac transthyretin amyloidosis. Circulation 133:2404–2412

    Article  CAS  Google Scholar 

  3. Lefaucheur JP, Ng Wing Tin S, Kerschen P et al (2013) Neurophysiological markers of small fibre neuropathy in TTR-FAP mutation carriers. J Neurol 260:1497–1503

    Article  Google Scholar 

  4. Kollmer J, Sahm F, Hegenbart U et al (2017) Sural nerve injury in familial amyloid polyneuropathy: MR neurography vs clinicopathologic tools. Neurology 89:475–484

    Article  CAS  Google Scholar 

  5. Kollmer J, Hund E, Hornung B et al (2015) In vivo detection of nerve injury in familial amyloid polyneuropathy by magnetic resonance neurography. Brain 138:549–562

    Article  Google Scholar 

  6. Misu K, Hattori N, Nagamatsu M et al (1999) Late-onset familial amyloid polyneuropathy type I (transthyretin Met30-associated familial amyloid polyneuropathy) unrelated to endemic focus in Japan: Clinicopathological and genetic features. Brain 122:1951–1962

    Article  Google Scholar 

  7. Masuda T, Ueda M, Kitajima M et al (2018) Teaching NeuroImages: morphology of lumbosacral dorsal root ganglia and plexus in hereditary transthyretin amyloidosis. Neurology 91:1834–1835

    Article  Google Scholar 

  8. Planté-Bordeneuve V, Gorram F, Salhi H et al (2017) Long-term treatment of transthyretin familial amyloid polyneuropathy with tafamidis: a clinical and neurophysiological study. J Neurol 264:268–276

    Article  Google Scholar 

  9. Tazawa K, Matsuda M, Yoshida T et al (2008) Spinal nerve root hypertrophy on MRI: clinical significance in the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy. Intern Med 47:2019–2024

  10. Chazen JL, Cornman-Homonoff J, Zhao Y et al (2018) MR neurography of the lumbosacral plexus for lower extremity radiculopathy: frequency of findings, characteristics of abnormal intraneural signal, and association with electromyography. AJNR Am J Neuroradiol 39:2154–2160

  11. Friedman J, Hastie T, Tibshirani R (2010) Regularization paths for generalized linear models via coordinate descent. J Stat Softw 33:1–22

    Article  Google Scholar 

  12. Good P (1994) Permutation tests: a practical guide to resampling methods for testing hypotheses. Springer Series in Statistics. Springer, New York. https://doi.org/10.1007/978-1-4757-2346-5

  13. Benjamini Y, Hochberg Y (1995) Controlling the false discovery rate: a practical and powerful approach to multiple testing. J R Stat Soc Ser B Methodol 57:289–300

    Google Scholar 

  14. Koike H, Katsuno M (2020) Transthyretin amyloidosis: update on the clinical spectrum, pathogenesis, and disease-modifying therapies. Neurol Ther 9:317–333

    Article  Google Scholar 

  15. Kronlage M, Schwehr V, Schwarz D et al (2019) Prevalence of fascicular hyperintensities in peripheral nerves of healthy individuals with regard to cerebral white matter lesions. Eur Radiol 29:3480–3487

    Article  Google Scholar 

  16. Jomier F, Bousson V, Viala K et al (2020) Prospective study of the additional benefit of plexus magnetic resonance imaging in the diagnosis of chronic inflammatory demyelinating polyneuropathy. Eur J Neurol 27:181–187

    Article  CAS  Google Scholar 

  17. Leonardi L, Vanoli F, Fionda L et al (2020) Nerve ultrasonography findings as possible pitfall in differential diagnosis between hereditary transthyretin amyloidosis with polyneuropathy and chronic inflammatory demyelinating polyneuropathy. Neurol Sci 41:3775–3778

    Article  Google Scholar 

  18. Chhabra A, Andreisek G, Soldatos T et al (2011) MR neurography: past, present, and future. AJR Am J Roengenol 197:583–591

Download references

Funding

The authors state that this work has not received any funding.

Author information

Authors and Affiliations

  1. Department of Radiology, Groupe Hospitalier Paris Saint-Joseph, Centre d’Imagerie Médicale Léonard de Vinci, Paris, France

    Jérôme Hodel

  2. Department of Neuroradiology, Henri Mondor University Hospital, Créteil, France

    Samir Benadjaoud

  3. Radiobiology and Regenerative Medicine Research Service, Institute for Radiological Protection and Nuclear Safety (IRSN), Fontenay-aux-Roses, France

    Mohamed Amine Benadjaoud

  4. EA 4391 and Clinical Neurophysiology Unit, Faculty of Health and Henri Mondor University Hospital, University Paris-Est Creteil and AP-HP , Créteil, France

    Jean-Pascal Lefaucheur

  5. Department of Neurology - Amyloid network, Henri Mondor University Hospital, AP-HP, University Paris-Est Créteil, INSERM, IMRB, Créteil, France

    Violaine Planté-Bordeneuve

Authors
  1. Jérôme Hodel
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Samir Benadjaoud
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Mohamed Amine Benadjaoud
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jean-Pascal Lefaucheur
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Violaine Planté-Bordeneuve
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jérôme Hodel.

Ethics declarations

Guarantor

The scientific guarantor of this publication is Prof Violaine Planté-Bordeneuve.

Conflict of interest

The authors declare no competing interests.

Statistics and biometry

One of the authors has significant statistical expertise. No complex statistical methods were necessary for this paper.

Informed consent

Written informed consent was obtained from all subjects (patients) in this study.

Ethical approval

Institutional Review Board approval was obtained.

Methodology

• prospective

• diagnostic or prognostic study

• performed at one institution

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hodel, J., Benadjaoud, S., Benadjaoud, M.A. et al. 3-T MR neurography of lumbo-sacral plexus in hereditary transthyretin-related amyloidosis with polyneuropathy. Eur Radiol 32, 7865–7871 (2022). https://doi.org/10.1007/s00330-022-08748-w

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00330-022-08748-w

Keywords

Search

Navigation