Banca de QUALIFICAÇÃO: ANA CRISTINA DE MEDEIROS GARCIA MACIEL

Uma banca de QUALIFICAÇÃO de DOUTORADO foi cadastrada pelo programa.
STUDENT : ANA CRISTINA DE MEDEIROS GARCIA MACIEL
DATE: 05/12/2024
TIME: 14:00
LOCAL: Remoto
TITLE:

Amyotrophic lateral sclerosis, respiratory muscles and mortality

KEY WORDS:

myoAtrophic lateral sclerosis - Respiratory Function Tests - Mortality

PAGES: 68
BIG AREA: Ciências da Saúde
AREA: Fisioterapia e Terapia Ocupacional
SUMMARY:

Introduction: Amyotrophic lateral sclerosis (ALS) is one of the main forms of degenerative motor neuron disease, clinically defined by the involvement of upper and/or lower motor neurons, leading to muscle weakness, disability, and respiratory failure, typically progressing to death. Respiratory impairment at various stages of the disease is well-known, and early and periodic assessment of respiratory function is mandatory. In recent decades, much attention has been devoted to studying variables that can predict early respiratory muscle weakness in these patients, as well as identifying factors that directly impact ALS functionality and mortality.

Objective: Article 1: To analyze and compare the electromyographic activity of respiratory muscles during SNIP and SNEP maneuvers in the seated position and at a 45° inclined position in ALS patients and a matched group of healthy subjects. Article 2: To investigate the relationship between respiratory function and mortality in ALS patients, identifying which respiratory parameters show the strongest correlation with clinical outcomes, specifically death.

Methods: Article 1: A descriptive observational study involving male subjects with ALS and matched healthy individuals. The evaluations included spirometry, surface electromyography (sEMG) of the sternocleidomastoid, scalene, rectus abdominis, and external oblique muscles during maximal respiratory pressure maneuvers (PImax and PEmax) in the seated position, and nasal respiratory pressures (SNIP and SNEP) in the seated and 45° inclined positions (randomized). Article 2: A retrospective study of individuals with ALS evaluated at the Pneumocardiovascular Laboratory and followed by a multidisciplinary team at the Neuromuscular Disease Clinic of HUOL/EBSERH from 2018 to 2023. Assessments included pulmonary function tests (spirometry, manovacuometry, and SNIP) and the ALS Functional Rating Scale (ALSFRS-r). Information on mortality was recorded during the period.

Results: Article 1: Twenty male individuals were evaluated, including 9 healthy controls (43.6 ± 11.6 years) and 11 with ALS (46.8 ± 12.5 years). In ALS patients, baseline electromyographic activity of the sternocleidomastoid muscle (SCM) at rest and during SNIP was higher than in healthy individuals in both positions (p < 0.05). No significant differences were found in the electrical activity of the other muscles or variables. SNEP values in the inclined position were lower than in the seated position in ALS patients (70.3 ± 26.7 vs. 57.3 ± 22.8 cmH₂O, p < 0.05). SNIP and SNEP were lower in ALS patients in the 45° inclined position compared to healthy controls (69.1 ± 2.72 vs. 95.5 ± 23.5 cmH₂O; 57.3 ± 22.5 vs. 92.7 ± 26.4 cmH₂O, p < 0.05, respectively). Article 2: Variables associated with mortality were FVC% predicted (rpb = -0.260; p < 0.001), SNIP (rpb = -0.235; p = 0.001), PEmax (rpb = -0.207; p = 0.007), PImax (rpb = -0.198; p = 0.009), and PEF% predicted (rpb = -0.156; p = 0.013). Variables related to ALSFRS-r were FVC% predicted (r = 0.621; p < 0.001), SNIP (r = 0.617; p < 0.001), PImax (r = 0.513; p < 0.001), PEmax (r = 0.511; p < 0.001), and PEF% predicted (r = 0.322; p < 0.001). A reduction of one unit in the respiratory variables PEF% predicted, PImax, and SNIP increased the risk of death by an average of 300% (OR = 2.99; 95% CI: 2.05-4.35), 2% (OR = 1.02; 95% CI: 1.01-1.03), and 1% (OR = 1.01; 95% CI: 1.00-1.02), respectively. It was not possible to establish a cutoff point for PEF, as the ROC curve's AUC was very low despite being significant (AUC = 0.66; p = 0.013).

Conclusion: The electromyographic activity of respiratory muscles in ALS is influenced by patient positioning, with higher SCM activity compared to other muscles. Lower SNIP and SNEP values were observed in the 45° inclined posture in ALS subjects compared to healthy individuals. Additionally, the progressive decline in SNIP and PEF values over time are predictors of mortality and functional decline.

COMMITTEE MEMBERS:
Interna - 3358154 - GIANE AMORIM RIBEIRO SAMORA
Presidente - 1545315 - GUILHERME AUGUSTO DE FREITAS FREGONEZI
Interna - 5566309 - VANESSA REGIANE RESQUETI FREGONEZI