Banca de DEFESA: ANA CRISTINA DE MEDEIROS GARCIA MACIEL

Uma banca de DEFESA de DOUTORADO foi cadastrada pelo programa.
STUDENT : ANA CRISTINA DE MEDEIROS GARCIA MACIEL
DATE: 25/07/2025
TIME: 09:00
LOCAL: HÍBRIDO
TITLE:

Amyotrophic lateral sclerosis, respiratory muscles and mortality

KEY WORDS:

Amyotrophic lateral sclerosis - Respiratory Function Tests - Mortality

PAGES: 95
BIG AREA: Ciências da Saúde
AREA: Fisioterapia e Terapia Ocupacional
SUMMARY:

Introduction: Amyotrophic Lateral Sclerosis (ALS) is characterized as a heterogeneous and fatal neurodegenerative disorder involving the degeneration of both upper and lower motor neurons in the brain and spinal cord. The impact of pulmonary function impairment, respiratory muscle strength, and functionality throughout the disease's progression, as well as their correlation with patient mortality, remains insufficiently understood despite the critical role of respiratory function in the survival of individuals with ALS. Additionally, monitoring respiratory muscle strength in different body positions can provide clinically significant information to guide early decisions regarding ventilatory support. Measurements of maximal inspiratory and expiratory pressures, as well as nasal respiratory pressures (SNIP and SNEP), are particularly relevant methods for ensuring adequate monitoring of ALS patients. Objectives: 1) To investigate the relationship between respiratory function, funcionality and mortality in ALS patients, identifying which respiratory parameters correlate most strongly with the clinical outcome of death. 2) To evaluate differences in SNIP and SNEP respiratory muscle peak pressures measured in two different positions (seated and supine with a 45° elevation) in ALS patients compared to a matched healthy control group. Additionally, to analyze and compare the electromyographic activity of inspiratory and expiratory muscles during respiratory pressure maneuvers in both positions. Materials and Methods: 1) A retrospective cohorte conducted between January 2018 and December 2023, involving ALS patients under outpatient follow-up. Clinical, anthropometric, respiratory, and functional evaluations were performed during routine outpatient visits. 2) A cross-sectional observational study involving ALS patients and a matched healthy control group. Participants underwent spirometry and surface electromyography (sEMG) of the sternocleidomastoid, scalene, rectus abdominis, and external oblique muscles during maximal respiratory pressure maneuvers (MIP and MEP) in the seated position, as well as nasal respiratory pressures (SNIP and SNEP) in seated and 45° inclined positions (randomized). Results: 1) Data from 74 ALS patients (mean age 55.70 ± 13.52 years; 66.2% male; predominantly spinal-onset ALS in 51.3%) were analyzed. Respiratory variables, except for peak expiratory flow (PEF), showed a weak but significant inverse correlation with mortality. All respiratory variables correlated positively (weak to moderate strength) and significantly with functionality as assessed by ALSFRS-R. A one-unit decrease in PEF%pred, MIP, and SNIP increased mortality risk by 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. 2) Twenty male participants were evaluated, including nine healthy controls (mean age 43.6 ± 11.6 years) and 11 ALS patients (mean age 46.8 ± 12.5 years). In ALS patients, SNEP values in the inclined position were lower than in the seated position (70.3 ± 26.7 vs. 57.3 ± 22.8 cmH₂O, p<0.05). Both SNIP and SNEP values were lower in ALS patients in the 45° inclined position compared to healthy controls (SNIP: 69.1 ± 27.2 vs. 95.5 ± 23.5 cmH₂O; SNEP: 57.3 ± 22.5 vs. 92.7 ± 26.4 cmH₂O; p<0.05). In ALS patients, resting basal electromyographic activity of the sternocleidomastoid was higher than in healthy individuals in both positions (p<0.05). No significant differences were found in electrical activity for other variables or measurements. Conclusions: In spinal-onset ALS, nasal pressures may be compromised due to reduced diaphragm and abdominal muscle efficacy in the supine position. The increased electrical activity of the sternocleidomastoid muscle in the 45° inclined position compared to controls may reflect compensatory weakness. Methodological rigor is crucial for optimal SNIP/SNEP testing performance. Retrospective study data suggest that respiratory muscle strength and function, particularly PEF and SNIP measurements, may serve as valuable markers to inform early interventions such as bronchial hygiene therapy and ventilatory support, improving quality of life and potentially extending survival.

COMMITTEE MEMBERS:
Presidente - 1545315 - GUILHERME AUGUSTO DE FREITAS FREGONEZI
Interna - 3358154 - GIANE AMORIM RIBEIRO SAMORA
Interna - 5566309 - VANESSA REGIANE RESQUETI FREGONEZI
Externa à Instituição - ANA ALINE MARCELINO DA SILVA - ESTACIO
Externa à Instituição - JESSICA DANIELLE MEDEIROS DA FONSECA - EBSERH