Background Bulbar involvement is a hallmark of amyotrophic lateral sclerosis (ALS), but surprisingly very few studies have addressed the frequency, pattern and clinical relevance of laryngeal involvement in the disease.
Methods Twenty-six patients with spinal-onset ALS underwent nasofibroscopy (NF), followed by laryngeal electromyography (LEMG). We also studied resting activity and motor unit potentials of the genioglossus and masseter muscles.
Results Twenty-four patients presented neurogenic changes in at least one laryngeal muscle. There were fibrillation and/or fasciculation potentials associated with chronic neurogenic changes in the same muscle in 16 patients; of these, 9 had no alteration in the genioglossus. We found no patient with tongue neurogenic changes and normal LEMG. NF was abnormal in 14 patients; in the remaining 12, LEMG identified neurogenic changes in 11 of them.
Conclusion LEMG is able to identify laryngeal denervation in patients with ALS, sometimes before clinical manifestations are noticed. This technique may be a useful diagnostic tool for selected patients with suspicion of ALS.
- motor neuron disease
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Contributors MPM and MCFJ had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: MPM, ANC, PATK, AN, MC and MCFJ. Acquisition, analysis and interpretation of data: all authors. Drafting the manuscript: MPM, ANC, PATK, MC and MCFJ. Critical revision of the manuscript for important intellectual content: all authors. Statistical analysis: MPM and MCFJ.
Funding MPM receives a PhD scholarship from CAPES (Coordenação de Aperfeiçoamento de pessoal de nível superior-BRAZIL). The funding agency did not interfere with the design of the study, collection of data or drafting of the manuscript.
Competing interests None declared.
Patient consent for publication Not required.
Ethics approval The study was approved by the local research ethics committee (CAAE 64254416.0.0000.5404).
Provenance and peer review Not commissioned; externally peer reviewed.
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