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Lingual fasciculation: A point of call for the diagnosis of amyotrophic lateral sclerosis.

ABSTRACT: A 60-year-old female patient, with no notable medical history, was referred by the internal medicine department for a dry mouth workup. The clinical examination revealed an absence of dryness, and the presence of lingual fasciculations, associated with difficulties in mastication and phonation. These symptoms appeared spontaneously 9 months before the consultation, after leaving confinement. Given the presence of lingual fasciculations, the diagnostic hypothesis of a neurological pathology, in particular amyotrophic lateral sclerosis (ALS), was suspected. After performing an electromyogram (EMG), the diagnosis of ALS was retained. Riluzole treatment was then started, and physical therapy sessions were scheduled. Riluzole allows an average gain of 4 to 6 months of life expectancy. Speech therapy and physical therapy allow to maintain the functions as long as possible and to improve the end-of-life conditions. The interest of early detection of ALS allows delaying the progression of the disease.

SUBMITTER: Galeazzi L

PROVIDER: S-EPMC10276246 | biostudies-literature | 2023 Jun

REPOSITORIES: biostudies-literature

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Lingual fasciculation: A point of call for the diagnosis of amyotrophic lateral sclerosis.

Galeazzi L L Holzman J J Mondoloni M M Rochefort J J

Clinical case reports 20230617 6

A 60-year-old female patient, with no notable medical history, was referred by the internal medicine department for a dry mouth workup. The clinical examination revealed an absence of dryness, and the presence of lingual fasciculations, associated with difficulties in mastication and phonation. These symptoms appeared spontaneously 9 months before the consultation, after leaving confinement. Given the presence of lingual fasciculations, the diagnostic hypothesis of a neurological pathology, in p ...[more]

PMID: 37334341

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Project description:BackgroundAlthough reduced lingual strength is a confirmed early manifestation of amyotrophic lateral sclerosis (ALS), its functional impact on swallowing remains unclear. We therefore sought to examine relationships between maximum anterior isometric lingual pressure (MAIP) with swallowing safety, swallowing efficiency, and swallowing timing metrics in a large cohort of individuals with ALS.MethodsNinety-seven participants with ALS completed a standardized videofluoroscopic swallowing examination (VF) and lingual pressure testing (Iowa Oral Performance Instrument). Duplicate and blinded ratings of the Penetration-Aspiration Scale (PAS) and Analysis of Swallowing Physiology: Events, Kinematics and Timing (ASPEKT) percent efficiency (%C2-C42 ) and timing (laryngeal vestibule closure (LVC) duration: amount of time (milliseconds, msec) between LVC onset and laryngeal vestibule opening; time-to-LVC: hyoid burst to onset of LVC (msec); and swallow reaction time: interval between bolus passing ramus of mandible and onset of LVC (msec)) were performed across bolus trials. Swallowing safety (safe PAS: 1, 2, 4; unsafe PAS: 3, 5, 6, 7, and 8) and efficiency (inefficient: ≥3% worst total residue) were derived. Statistical analyses including descriptives, binary logistic regressions, and Spearman's rho correlations were performed (α = 0.05).Key resultsMean MAIP was 36.3 kPa (SD: 18.7). Mean MAIP was higher in those with safe swallowing as compared to those who penetrated (mean difference: 12 kPa) or aspirated (mean difference: 18 kPa). Individuals with efficient swallowing demonstrated higher MAIP than those with inefficient swallowing (mean difference: 11 kPa). Binary logistic regression analyses revealed increasing MAIP was significantly associated with a 1.06 (95% CI: 1.03-1.09) and 1.04 (95% CI: 1.01-1.06) greater odds of safe and efficient swallowing, respectively. No relationships were observed between MAIP and swallow reaction time across all bolus trials. Longer time-to-LVC (5 ml thin liquid: rs = -0.35, p = 0.002; cup sip thin liquid: rs = -0.26, p = 0.02; moderately thick liquid: rs = -0.28, p = 0.01) and prolonged LVC duration (cup sip thin liquid, rs = -0.34, p = 0.003) were associated with lower MAIP.Conclusions and inferencesReduced lingual strength was confirmed in this group of 97 individuals with ALS that was associated with a diminished ability to effectively transport boluses and aide in laryngeal vestibule closure to prevent entry of material into the airway.

Project description:IntroductionAmyotrophic lateral sclerosis (ALS) is a rare, devastating neurodegenerative disease that affects upper and lower motor neurons. To date, no effective treatment or reliable biomarker for ALS has been developed. In recent years, many factors have been proposed as possible biomarkers of ALS; however, no consensus has been reached. Therefore, a reliable biomarker is urgently needed. Eosinophils may play a crucial role in healthy humans and diseases, and serve as a biomarker for many chronic diseases.MethodsRoutine blood test results were collected from 66 healthy controls and 59 patients with ALS. The percentages and total numbers of each cell population were analyzed, and the correlation between these indicators and patient ALS functional rating scale-revised (ALSFRS-R) score or disease progression rate (ΔFS score) was analyzed.ResultsCompared to healthy controls, the number of blood leukocytes, neutrophils, monocytes, and basophils was significantly decreased in patients with ALS (p = 0.002, p = 0.001, p = 0.049, and p < 0.0001, respectively). There was an increase in the number of eosinophils (p < 0.0001), but no difference in the number of lymphocytes between patients with ALS and healthy controls was found (p = 0.563). Compared to healthy controls, the percentage of neutrophils was decreased and the percentage of lymphocytes and eosinophils was increased in patients with ALS (p = 0.01, p = 0.012, and p = 0.001, respectively). There was no difference between patients with ALS and healthy controls in the percentage of monocytes and basophils (p = 0.622 and p = 0.09, respectively). However, only the percentage and number of eosinophils had a correlation with the ΔFS score. Further multivariate analysis revealed a significant correlation between the disease duration, eosinophil count and percentage, and the disease progression rate (p < 0.0001, p = 0.048, and p = 0.023, respectively). The neutrophil-to-eosinophil ratio (NER), lymphocyte-to-eosinophil ratio (LER), and monocyte-to-eosinophil ratio (MER) were significantly lower in patients with ALS than in healthy controls. However, only the LER was significantly correlated with the ΔFS score.ConclusionThese observations implicate neutrophils, lymphocytes, and eosinophils as important factors, and increasing eosinophil counts were negatively correlated with the ΔFS score in patients with ALS.

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Lingual fasciculation: A point of call for the diagnosis of amyotrophic lateral sclerosis.

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Lingual fasciculation: A point of call for the diagnosis of amyotrophic lateral sclerosis.

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