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Nerve conduction study on the split-hand plus index in Amyotrophic lateral sclerosis: correlations with lower motor neuron impairment

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Abstract

Introduction

In the arms of patients with Amyotrophic lateral sclerosis (ALS) two peculiar patterns of dissociated muscular atrophy have been described: the split-hand sign (with predominant atrophy of the lateral aspect of the hand, compared to hypothenar eminence) and the split-hand-plus sign (SHPS), a predominant abductor pollicis brevis (ABP) atrophy with sparing of flexor pollicis longus (FPL).

Aims

In this case–control study, we evaluated the diagnostic utility of a neurophysiological indicator of SHPS and assessed its association with clinical features.

Methods

We prospectively studied 59 incident ALS patients, 61 patients with ALS-mimic disorders (OND) and 61 non-neurological controls (NNCs). ABP and FPL compound muscle action potentials (CMAP) amplitudes were obtained by supramaximal stimulation of median nerve at elbow. Split-hand plus index (SHPI) was calculated according to the formula: APB-CMAP/FPL-CMAP.

Results

SHPI was significantly lower in ALS compared to OND patients and NNCs (p < 0.0001). SHPI value < 1 was observed in 2% of NNCs and 9% of OND patients and demonstrated an accuracy of 71% in differentiating ALS from OND and an accuracy of 74% in differentiating ALS from NNC. SHPI was associated with higher LMN score, and higher disease severity as quantified by the ALSFRS-r.

Conclusion

Our results indicate that SHPI is a reliable indicator to distinguish ALS patients from ONDs and NNCs. SHPI was significantly associated to the degree of lower motor neuron impairment but showed no association with upper motoneuron impairment.

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Funding

The author(s) declare financial support was received for the research, authorship, and/or publication of this article. This work was supported by Regione Puglia and CNR for Tecnopolo per la Medicina di Precisione. D.G.R. n. 2117 of 21.11.2018 (CUPB84I18000540002)—C.I.R.E.M.I.C. (Research Center of Excellence for Neurodegenerative Diseases and Brain Aging)—University of Bari Aldo Moro.

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Authors and Affiliations

  1. Operative Unit of Neurology, San Paolo Hospital, ASL Bari, Via Caposcardicchio snc, Bari, Italy

    Stefano Zoccolella, Vito Devitofrancesco, Rosaria Damato, Fulvia Piomboni & Salvatore Misceo

  2. Center for Neurodegenerative Disease and The Aging Brain, University of Bari Aldo Moro at Pia Fondazione “Cardinale G. Panico” Tricase, Lecce, Italy

    Stefano Zoccolella, Alessia Giugno, Marco Filardi, Ludovica Tamburrino & Giancarlo Logroscino

  3. Department of Translational Biomedicine and Neurosciences (DiBraiN), University of Bari Aldo Moro, Bari, Italy

    Giammarco Milella, Marco Filardi, Eustachio D’Errico, Ludovica Tamburrino & Giancarlo Logroscino

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  1. Stefano Zoccolella
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  2. Giammarco Milella
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Corresponding author

Correspondence to Stefano Zoccolella.

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Ethical statement

Approval for this study was granted by the Ethics Committee for Medical Research at Azienda Sanitaria Locale Lecce on May 25, 2017 (approval number 6), ensuring compliance with the ethical standards outlined in the Helsinki Declaration.

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Zoccolella, S., Milella, G., Giugno, A. et al. Nerve conduction study on the split-hand plus index in Amyotrophic lateral sclerosis: correlations with lower motor neuron impairment. Neurol Sci (2024). https://doi.org/10.1007/s10072-024-07582-w

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