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.
Nerve ultrasound ALS MMN Diagnosis Nerve conduction studies
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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.
Compliance with ethical standards
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.
The study was approved by the responsible Ethics Review Committee at the Technische Universität Dresden, Germany.
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