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Nerve ultrasound for differentiation between amyotrophic lateral sclerosis and multifocal motor neuropathy

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Abstract

Ultrasound is useful for non-invasive visualization of focal nerve pathologies probably resulting from demyelination, remyelination, edema or inflammation. In patients with progressive muscle weakness, differentiation between multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis (ALS) is essential regarding therapy and prognosis. Therefore, the objective of this study was to investigate whether nerve ultrasound can differentiate between ALS and MMN. Systematic ultrasound measurements of peripheral nerves and the 6th cervical nerve root (C6) were performed in 17 patients with ALS, in 8 patients with MMN and in 28 healthy controls. Nerve conduction studies of corresponding nerves were undertaken in MMN and ALS patients. Electromyography was performed in ALS patients according to revised El-Escorial criteria. ANOVA and unpaired t test with Bonferroni correction revealed significant differences in cross-sectional areas (CSA) of different nerves and C6 diameter between the groups. Nerve enlargement was found significantly more frequently in MMN than in other groups (p < 0.001). Receiver operating characteristics analysis revealed detection of enlarged nerves/roots in at least four measurement points to serve as a good marker to differentiate MMN from ALS with a sensitivity of 87.5 % and a specificity of 94.1 %. Ultrasonic focal nerve enlargement in MMN was often not colocalized with areas of conduction blocks found in nerve conduction studies. Systematic ultrasound measurements in different nerves and nerve roots are valuable for detecting focal nerve enlargement in MMN, generally not found in ALS and thus could serve as a diagnostic marker to differentiate between both entities in addition to electrodiagnostic studies.

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Abbreviations

ALS:

Amyotrophic lateral sclerosis

AUC:

Area under the curve

CIDP:

Chronic inflammatory demyelinating polyradiculoneuropathy

CMAP:

Compound muscle action potential

CMT:

Charcot Marie Tooth

CSA:

Cross-sectional area

CV:

Conduction velocity

C6:

Cervical nerve root 6

EFNS:

European Federation of Neurological Societies/Peripheral Nerve Society

GBS:

Guillain-Barré syndrome

ICC:

Intraclass correlation coefficient

ICSAV:

Intranerve CSA variability

LMN:

Lower motor neuron

MMF:

Mycophenolatmofetil

MMN:

Multifocal motor neuropathy

NCS:

Nerve conduction studies

PNP:

Polyneuropathy

PNUS:

Peripheral nerve ultrasound

PSW:

Positive sharp waves

ROC:

Receiver operating characteristics

SNAP:

Sensory nerve action potential

UMN:

Upper motor neuron

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Acknowledgments

The research was supported by the German Center for Sepsis Control and Care (CSCC, funded by the Ministry of Education and Research (BMBF), Grant No 01 E0 1002. The authors thank Nasim Kroegel for her help in language editing.

Conflicts of interest

The authors declare that they have no conflict of interest.

Ethical standards

The study was registered in the German Clinical Trials Register (DRKS-ID: DRKS0005253) and approved by the local ethics committee (No. 3663-01/13 and EKZN 2014-230). Informed consent was obtained from all patients and controls.

Author information

Authors and Affiliations

  1. Department of Neurology, Neuromuscular Center, Basel University Hospital, University Basel, Petersgraben 4, 4000, Basel, Switzerland

    Alexander Grimm, Bernhard F. Décard, Ioanna Athanasopoulou, Kathi Schweikert & Michael Sinnreich

  2. Hans Berger Department of Neurology, Jena University Hospital, Jena, Germany

    Hubertus Axer

  3. Center for Sepsis Control and Care (CSCC), Jena University Hospital, Jena, Germany

    Hubertus Axer

Authors
  1. Alexander Grimm
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  2. Bernhard F. Décard
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  3. Ioanna Athanasopoulou
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  4. Kathi Schweikert
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  5. Michael Sinnreich
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  6. Hubertus Axer
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Correspondence to Alexander Grimm.

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Grimm, A., Décard, B.F., Athanasopoulou, I. et al. Nerve ultrasound for differentiation between amyotrophic lateral sclerosis and multifocal motor neuropathy. J Neurol 262, 870–880 (2015). https://doi.org/10.1007/s00415-015-7648-0

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  • DOI: https://doi.org/10.1007/s00415-015-7648-0

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