Skip to main content
Log in

Exploring the Split-Hand Phenomenon in ALS with Motor unit Number Index (MUNIX)

  • Published:
Human Physiology Aims and scope Submit manuscript

Abstract

Split-hand phenomenon in amyotrophic lateral sclerosis (ALS) is characterized by uneven progression of muscle weakness in the intrinsic hand muscles, with predominant wasting of abductor pollicis brevis (APB) and first dorsal interosseus muscles over abductor digiti minimi (ADM) muscle. The aim of the study was to evaluate the split-hand phenomenon in ALS patients using motor unit index (MUNIX). Fifty-six patients diagnosed with definite and probable ALS according to El Escorial criteria were enrolled in the study. Twenty-seven patients (48.2%) had bulbar-onset ALS, while twenty-nine patients (51.8%) presented with spinal-onset ALS. Clinical measures including disease duration, disease stage according to the King’s College staging system, and ALSFRS-R score were evaluated for each patient. The control group consisted of twenty-two healthy subjects with normal findings on nerve conduction studies of the upper extremities. Both APB and ADM were studied, and ratios (APB/ADM) of the compound muscle action potential (CMAP) and MUNIX were assessed. Although CMAP and MUNIX ratios were lower in ALS than in healthy controls, this reached statistical significance only for the MUNIX ratio. No correlations were found between electrophysiological data (MUNIX and CMAP ratios) and clinical measures in the ALS group, which could be caused by high clinical heterogeneity in the ALS group. Our findings correspond to the literature data on high diagnostic sensitivity of MUNIX in assessing the split-hand phenomenon. MUNIX is a promising tool for evaluation of the split-hand phenomenon, which can be widely used both for analyzing mechanisms of disease pathology in longitudinal studies and improving early diagnosis of the disease.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1.
Fig. 2.
Fig. 3.
Fig. 4.

Similar content being viewed by others

REFERENCES

  1. Masrori, P. and van Damme, P., Amyotrophic lateral sclerosis: a clinical review, Eur. J. Neurol., 2020, vol. 27, no. 10, p. 1918.

    Article  CAS  Google Scholar 

  2. Ludolph, A.C., et al., Pattern of paresis in ALS is consistent with the physiology of the corticomotoneuronal projections to different muscle groups, J. Neurol., Neurosurg. Psychiatry, 2020, vol. 91, no. 9, p. 991.

    Article  Google Scholar 

  3. Wang, Z.-L., et al., A prospective study on split-hand index as a biomarker for the diagnosis of amyotrophic lateral sclerosis, Amyotrophic Lateral Scler. Frontotemporal Degener., 2020, vol. 21, nos. 7–8, p. 574.

    Article  Google Scholar 

  4. Abraham, A., et al., Split-hand phenomenon in motor neuron diseases: sonographic assesment of muscle thickness, Clin. Neurophysiol., 2020, vol. 131, no. 8, p. 1721.

    Article  Google Scholar 

  5. Wang, Z.-L., et al., Split-hand index in amyotrophic lateral sclerosis: an F-wave study, Amyotrophic Lateral Scler. Frontotemporal Degener., 2019, vol. 20, no. 7, p. 562.

    Article  Google Scholar 

  6. Bostock, H., Jacobsen, A.B., and Tankisi, H., Motor unit number index and compound muscle action potential amplitude, Clin. Neurophysiol., 2019, vol. 130, no. 9, p. 1734.

    Article  CAS  Google Scholar 

  7. Nandedkar, S.D., et al., Motor unit number index (MUNIX), IEEE Trans. Biomed. Eng., 2004, vol. 51, no. 12, p. 2209.

    Article  Google Scholar 

  8. Huynh, W., et al., Functional biomarkers for amyotrophic lateral sclerosis, Front. Neurol., 2019, vol. 9, p. 1141.

    Article  Google Scholar 

  9. Gunes, T., et al., Use of CMAP, MScan fit-MUNE, and MUNIX in understanding neurodegeneration pattern of ALS and detection of early motor neuron loss in daily practice, Neurosci. Lett., 2021, vol. 741, p. 135488.

    Article  CAS  Google Scholar 

  10. Fatehi, F., et al., The utility of motor unit number index: a systematic review, Neurophysiol. Clin., 2018, vol. 48, no. 5, p. 251.

    Article  Google Scholar 

  11. Swash, M., Reading the palm with MUNIX: a “reversed split hand” in spinal muscular atrophy, Clin. Neurophysiol., 2019, vol. 130, no. 2, p. 305.

    Article  Google Scholar 

  12. Zheng, C., et al., Split-hand phenomenon quantified by the motor unit number index for distinguishing cervical spondylotic amyotrophy from amyotrophic lateral sclerosis, Neurophysiol. Clin., 2019, vol. 49, no. 5, p. 391.

    Article  Google Scholar 

  13. Kim, D.G., et al., Split-hand phenomenon in amyotrophic lateral sclerosis: a motor unit number index study, Muscle Nerve, 2016, vol. 53, no. 6, p. 885.

    Article  Google Scholar 

  14. Corcia, P., et al., Split-hand and split-limb phenomena in amyotrophic lateral sclerosis: pathophysiology, electrophysiology and clinical manifestations, J. Neurol., Neurosurg. Psychiatry, 2021, vol. 92, no. 10, p. 1126.

    Article  Google Scholar 

  15. Swash, M., Reading the palm with MUNIX: A’reversed split hand’in spinal muscular atrophy, Clin. Neurophysiol., 2018, vol. 130, no. 2, p. 305.

    Article  Google Scholar 

  16. Singh, R.-J., et al., Reverse split hand syndrome: dissociated intrinsic hand muscle atrophy pattern in Hirayama disease/brachial monomelic amyotrophy, Amyotrophic Lateral Scler. Frontotemporal Degener., 2017, vol. 18, nos. 1–2, p. 10.

    Article  Google Scholar 

  17. Wang, Z.-L., et al., Split-hand syndrome in amyotrophic lateral sclerosis: differences in dysfunction of the FDI and ADM spinal motoneurons, Front. Neurosci., 2019, vol. 13, p. 371.

    Article  Google Scholar 

  18. Weber, M., et al., The split hand in ALS has a cortical basis, J. Neurol. Sci., 2000, vol. 180, no. 1, p. 66.

    Article  CAS  Google Scholar 

  19. Bae, J.S., et al., Cortical hyperexcitability and the split-hand plus phenomenon: pathophysiological insights in ALS, Amyotrophic Lateral Scler. Frontotemporal Degener., 2014, vol. 15, nos. 3–4, p. 250.

    Article  Google Scholar 

  20. Menon, P., et al., Sensitivity and specificity of threshold tracking transcranial magnetic stimulation for diagnosis of amyotrophic lateral sclerosis: a prospective study, Lancet Neurol., 2015, vol. 14, no. 5, p. 478.

    Article  Google Scholar 

  21. Menon, P., Kiernan, M.C., and Vucic, S., Cortical dysfunction underlies the development of the split-hand in amyotrophic lateral sclerosis, PLoS One, 2014, vol. 9, no. 1, p. e87124.

    Article  Google Scholar 

  22. de Carvalho, M. and Swash, M., The split hand in amyotrophic lateral sclerosis: a possible role for the neuromuscular junction, Amyotrophic Lateral Scler. Frontotemporal Degener., 2019, vol. 20, nos. 5–6, p. 368.

    Article  Google Scholar 

  23. Kim, D.G., et al., Split-hand phenomenon in amyotrophic lateral sclerosis: a motor unit number index study, Muscle Nerve, 2016, vol. 53, no. 6, p. 885.

    Article  Google Scholar 

  24. Günther, R., et al., Motor Unit Number Index (MUNIX) of hand muscles is a disease biomarker for adult spinal muscular atrophy, Clin. Neurophysiol., 2019, vol. 130, no. 2, p. 315.

    Article  Google Scholar 

  25. Zheng, C., et al., Split-hand phenomenon quantified by the motor unit number index for distinguishing cervical spondylotic amyotrophy from amyotrophic lateral sclerosis, Neurophysiol. Clin., 2019, vol. 49, no. 5, p. 391.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to A. A. Abramova.

Ethics declarations

The authors declare that there is no conflicts of interest.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Abramova, A.A., Broutian, A.G. & Zakharova, M.N. Exploring the Split-Hand Phenomenon in ALS with Motor unit Number Index (MUNIX). Hum Physiol 47, 854–860 (2021). https://doi.org/10.1134/S0362119721080016

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1134/S0362119721080016

Navigation