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European Radiology

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19-04-2022 | Polyneuropathy | Magnetic Resonance

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

Authors: Jérôme Hodel, Samir Benadjaoud, Mohamed Amine Benadjaoud, Jean-Pascal Lefaucheur, Violaine Planté-Bordeneuve

Published in: European Radiology | Issue 11/2022

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.

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Title: 3-T MR neurography of lumbo-sacral plexus in hereditary transthyretin-related amyloidosis with polyneuropathy
Authors: Jérôme Hodel
Samir Benadjaoud
Mohamed Amine Benadjaoud
Jean-Pascal Lefaucheur
Violaine Planté-Bordeneuve
Publication date: 19-04-2022
Publisher: Springer Berlin Heidelberg
Keywords: Polyneuropathy
Amyloidosis
Published in: European Radiology / Issue 11/2022
Print ISSN: 0938-7994
Electronic ISSN: 1432-1084
DOI: https://doi.org/10.1007/s00330-022-08748-w

At a glance: The STEP trials

Springer Medicine

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

Abstract

Objectives

Methods

Results

Conclusions

Key Points

At a glance: The STEP trials

Springer Medicine

Abstract

Objectives

Methods

Results

Conclusions

Key Points

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