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Introduction: Split phenomena in ALS refers to the preferential dysfunction of some groups of muscles over others. The split-elbow sign (SE) is characterized by the predominant weakness of the biceps compared to the triceps, but available results are conflicting.

Objectives: To evaluate the prevalence of the SE in two independent cohorts: the randomized controlled trial-based PRO-ACT cohort (n = 500) and a monocentric cohort of patients with ALS from Southern Italy (n = 144); to investigate the demographic and clinical variables associated with the SE sign.

Methods: Wilcoxon signed-rank test was used to compare biceps with triceps power in the same limb measured by hand-held dynamometry in the PRO-ACT cohort and Medical Research Council (MRC) in our cohort. Each limb was considered independently and not paired within the same individual. The arm where the triceps was stronger than the biceps was defined SE + , whereas the arm where the biceps was stronger than the triceps was considered SE-. A backward stepwise multivariate logistic regression was used to analyze the relationship between clinical and demographic variables and SE. PENN Upper Motor Neuron and Devine scales were used to evaluate the different upper (UMN) and lower (LMN) motor neuron impairments between the SE + and SE- arms.

Results: In both cohorts, the biceps were on average stronger than the triceps, and the SE sign was present in 41% of the PRO-ACT cohort and just 30% of the Southern Italy cohort. The multivariate logistic regression revealed that older age (OR: 1.45; p = 0.01), male gender (OR: 1.55; p = 0.002), spinal onset (OR: 1.59; p = 0.007), and higher disease severity (OR: 1.70; p = 0.001) were significant predictors of the SE sign in the PRO-ACT cohort. Conversely, in Southern Italy patients, only a lower ALSFRS-R score was a significant determinant of the SE (OR: 8.47; p = 0.008). Finally, SE + arms exhibited a significantly higher median Devine sub-score compared to SE- [1 vs 0, p = < 0.05], while arms SE- showed a significantly higher median PUMNS sub-score [2 vs 0; p = < 0.05)].

Conclusion: In our study, most patients with ALS do not show SE. Patients with SE are more likely older, males, with spinal onset, a higher degree of disease severity, and predominant and wider LMN impairment.

Titel:	Split-elbow sign in the PRO-ACT and Southern Italy ALS cohorts: a potential marker of disease severity and lower motor neuron involvement?
Autor/in / Beteiligte Person:	Zoccolella, S ; Milella, G ; Giugno, A ; Urso, D ; Tamburrino, L ; Nigro, S ; Gnoni, V ; Filardi, M ; Logroscino, G
Link:	View record at Springer (Volltext)
Zeitschrift:	Journal of neurology, Jg. 270 (2023-06-01), Heft 6, S. 3204-3212
Veröffentlichung:	Berlin ; New York, Springer-Verlag, 2023
Medientyp:	academicJournal
ISSN:	1432-1459 (electronic)
DOI:	10.1007/s00415-023-11660-y
Schlagwort:	Humans Male Elbow Motor Neurons Muscle, Skeletal Patient Acuity Amyotrophic Lateral Sclerosis diagnosis Amyotrophic Lateral Sclerosis epidemiology Amyotrophic Lateral Sclerosis complications Motor Neuron Disease
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Brain J Neurol 112(Pt 3):749–763. https://doi.org/10.1093/brain/112.3.749. (PMID: 10.1093/brain/112.3.749) Contributed Indexing: Keywords: Amyotrophic lateral sclerosis; Prognosis; Split phenomena; Split-elbow Entry Date(s): Date Created: 20230314 Date Completed: 20230518 Latest Revision: 20230518 Update Code: 20240513

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Split-elbow sign in the PRO-ACT and Southern Italy ALS cohorts: a potential marker of disease severity and lower motor neuron involvement?