Skip to main content

Advertisement

SpringerLink
Log in

Nerve ultrasound in the differentiation of multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis with predominant lower motor neuron disease (ALS/LMND)

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

Abstract

The objective of the study was to investigate nerve ultrasound (US) in comparison to nerve conduction studies (NCS) for differential diagnosis of amyotrophic lateral sclerosis with predominant lower motoneuron disease (ALS/LMND) and multifocal motor neuropathy (MMN). A single-center, prospective, examiner-blinded cross-sectional diagnostic study in two cohorts was carried out. Cohort I: convenience sample of subjects diagnosed with ALS/LMND or MMN (minimal diagnostic criteria: possible ALS (revised EL-Escorial criteria), possible MMN (European Federation of Neurosciences guidelines). Cohort II: consecutive subjects with suspected diagnosis of either ALS/LMND or MMN. Diagnostic US and NCS models were developed based on ROC analysis of 28 different US and 32 different NCS values measured in cohort I. Main outcome criterion was sensitivity/specificity of these models between ALS/LMND and MMN in cohort II. Cohort I consisted of 16 patients with ALS/LMND and 8 patients with MMN. For cohort II, 30 patients were recruited, 8 with ALS/LMND, 5 with MMN, and 17 with other diseases. In cohort I, the three best US measures showed higher mean ± SD areas under the curve than the respective NCS measures (0.99 ± 0.01 vs. 0.79 ± 0.03, p < 0.001; two-sided t test). The US model with highest measurement efficacy (8 values) and diagnostic quality reached 100 % sensitivity and 92 % specificity for MMN in cohort II, while the respective NCS model (6 values, including presence of conduction blocks) reached 100 and 52 %. Nerve US is of high diagnostic accuracy for differential diagnosis of ALS/LMND and MMN. It might be superior to NCS in the diagnosis of MMN in hospital-admitted patients with this differential diagnosis.

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

Similar content being viewed by others

References

  1. Ahdab R, Creange A, Saint-Val C, Farhat WH, Lefaucheur JP (2013) Rapidly progressive amyotrophic lateral sclerosis initially masquerading as a demyelinating neuropathy. Clin Neurophysiol 43:181–187

    Article  CAS  Google Scholar 

  2. Miyashiro A, Matsui N, Shimatani Y et al (2014) Are multifocal motor neuropathy patients underdiagnosed? An epidemiological survey in Japan. Muscle Nerve 49:357–361

    Article  PubMed  Google Scholar 

  3. Ravits J, Paul P, Jorg C (2007) Focality of upper and lower motor neuron degeneration at the clinical onset of ALS. Neurology 68:1571–1575

    Article  PubMed  Google Scholar 

  4. Ravits JM, La Spada AR (2009) ALS motor phenotype heterogeneity, focality, and spread: deconstructing motor neuron degeneration. Neurology 73:805–811

    Article  PubMed Central  PubMed  Google Scholar 

  5. Lin CS (2014) Multifocal motor neuropathy: lost in conduction block? J Neurol Neurosurg Psychiatry 85:1062

    Article  PubMed  Google Scholar 

  6. Delmont E, Azulay JP, Giorgi R et al (2006) Multifocal motor neuropathy with and without conduction block: a single entity? Neurology 67:592–596

    Article  CAS  PubMed  Google Scholar 

  7. Cetin H, Rath J, Fuzi J et al (2015) Epidemiology of amyotrophic lateral sclerosis and effect of Riluzole on disease course. Neuroepidemiology 44:6–15

    Article  PubMed  Google Scholar 

  8. van Schaik IN, Bouche P, Illa I et al (2006) European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy. Eur J Neurol 13:802–808

    Article  PubMed  Google Scholar 

  9. Cartwright MS, Hobson-Webb LD, Boon AJ et al (2012) Evidence-based guideline: neuromuscular ultrasound for the diagnosis of carpal tunnel syndrome. Muscle Nerve 46:287–293

    Article  PubMed  Google Scholar 

  10. Beekman R, van den Berg LH, Franssen H, Visser LH, van Asseldonk JT, Wokke JH (2005) Ultrasonography shows extensive nerve enlargements in multifocal motor neuropathy. Neurology 65:305–307

    Article  CAS  PubMed  Google Scholar 

  11. Rajabally YA, Morlese J, Kathuria D, Khan A (2012) Median nerve ultrasonography in distinguishing neuropathy sub-types: a pilot study. Acta Neurol Scand 125:254–259

    Article  CAS  PubMed  Google Scholar 

  12. Uncini A, Susuki K, Yuki N (2013) Nodo-paranodopathy: beyond the demyelinating and axonal classification in anti-ganglioside antibody-mediated neuropathies. Clin Neurophysiol 124:1928–1934

    Article  PubMed  Google Scholar 

  13. Taylor BV, Dyck PJB, Engelstad J, Gruener G, Grant I, Dyck PJ (2004) Multifocal motor neuropathy: pathologic alterations at the site of conduction block. J Neuropathol Exp Neurol 63:129–137

    Article  PubMed  Google Scholar 

  14. Cartwright MS, Walker FO, Griffin LP, Caress JB (2011) Peripheral nerve and muscle ultrasound in amyotrophic lateral sclerosis. Muscle Nerve 44:346–351

    Article  PubMed Central  PubMed  Google Scholar 

  15. Grimm A, Décard BF, Athanasopoulou I, Schweikert K, Sinnreich M, Axer H (2015) Nerve ultrasound for differentiation between amyotrophic lateral sclerosis and multifocal motor neuropathy. J Neurol 262(4):870–880

    Article  PubMed  Google Scholar 

  16. Brooks BR, Miller RG, Swash M, Munsat TL (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotrophic Lateral Scler Other Motor Neuron Disord 1:293–299

    Article  CAS  Google Scholar 

  17. Kleyweg RP, van der Meche FG, Schmitz PI (1991) Interobserver agreement in the assessment of muscle strength and functional abilities in Guillain-Barre syndrome. Muscle Nerve 14:1103–1109

    Article  CAS  PubMed  Google Scholar 

  18. Merkies IS, Lauria G (2006) 131st ENMC international workshop: selection of outcome measures for peripheral neuropathy clinical trials 10-12 December 2004, Naarden, The Netherlands. Neuromusc Disord NMD 16:149–156

    Article  CAS  PubMed  Google Scholar 

  19. Cedarbaum JM, Stambler N, Malta E et al (1999) The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci 169:13–21

    Article  CAS  PubMed  Google Scholar 

  20. Zaidman CM, Al-Lozi M, Pestronk A (2009) Peripheral nerve size in normals and patients with polyneuropathy: an ultrasound study. Muscle Nerve 40:960–966

    Article  PubMed  Google Scholar 

  21. Schreiber S, Abdulla S, Debska-Vielhaber G et al (2015) Peripheral nerve ultrasound in amyotrophic lateral sclerosis phenotypes. Muscle Nerve 51(5):669–675

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

The authors would like to express their thankfulness to the participating study subjects, especially those who are highly handicapped and still participated. We would also like to thank Martin Pette for his help in electrophysiology diagnostics, as well as Sabine Fitzthum and Max Waldmann, technical assistants very supportive of our study.

Author information

Authors and Affiliations

  1. Division of Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden, Fetscherstrasse 74, 01307, Dresden, Germany

    Kai F. Loewenbrück, Julia Liesenberg, Markus Dittrich, Jochen Schäfer, Andreas Hermann & Alexander Storch

  2. Department of Neurology, Elblandkliniken, 01662, Meissen, Germany

    Markus Dittrich

  3. Department of Neurology, Saxon Hospital Arnsdorf, 01477, Arnsdorf, Germany

    Beate Patzner & Beate Trausch

  4. Department of Neurology, Hospital Dresden Neustadt, 01129, Dresden, Germany

    Jochen Machetanz

  5. German Center for Neurodegenerative Diseases (DZNE), 18147, Rostock, Germany

    Andreas Hermann & Alexander Storch

  6. Department of Neurology, University of Rostock, Gehlsheimer Strasse 20, 18147, Rostock, Germany

    Alexander Storch

Authors
  1. Kai F. Loewenbrück
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Julia Liesenberg
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Markus Dittrich
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jochen Schäfer
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Beate Patzner
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Beate Trausch
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Jochen Machetanz
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Andreas Hermann
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Alexander Storch
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Alexander Storch.

Ethics declarations

Funding

This study was funded by the Bundesministerium für Bildung und Forschung (Federal Ministry for Education and Research Germany; AZ: 03WKBH2F) to A.S., and an unrestricted research grant from the Roland-Ernst Foundation to K.L. and A.S. The funders had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

Conflicts of interest

The authors report no conflict of interest.

Ethics approval

The study was approved by the responsible Ethics Review Committee at the Technische Universität Dresden, Germany.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary material 1 (DOCX 293 kb)

Supplementary material 2 (DOCX 22 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Loewenbrück, K.F., Liesenberg, J., Dittrich, M. et al. Nerve ultrasound in the differentiation of multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis with predominant lower motor neuron disease (ALS/LMND). J Neurol 263, 35–44 (2016). https://doi.org/10.1007/s00415-015-7927-9

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-015-7927-9

Keywords

Search

Navigation