Full text
| |
| Author(s): |
Vinícius Carlos Iamonti
Total Authors: 1
|
| Document type: | Doctoral Thesis |
| Press: | São Paulo. |
| Institution: | Universidade de São Paulo (USP). Faculdade de Medicina (FM/SBD) |
| Defense date: | 2023-04-17 |
| Examining board members: |
André Luiz Pereira de Albuquerque;
Pedro Caruso;
Frederico Mennucci de Haidar Jorge
|
| Advisor: | André Luiz Pereira de Albuquerque |
| Abstract | |
Introduction: Regardless of the onset site (bulbar or spinal) of the symptoms of motor neurodegeneration, the involvement of the respiratory system is a threat to the life of the patient with ALS, however, there might be a mismatch between the symptom of shortness of breath reported and ventilatory and functional test impaired. Objective: To compare lung function, ventilatory muscle strength and endurance capacity, quality of life, physical function and exercise capacity in oligosymptomatic patients with ALS and the control group. Evaluate the rate of disease progression after six months in the ALS group. Methods: Cross-sectional and prospective study involving 28 oligosymptomatic patients with ALS and 23 individuals from the control group. Shortness of breath was measured with (mMRC, BDI/TDI and ALSFRS-rcontrolled domain), quality of life (SF-36), functioning capacity and physical capacity level (accelerometer), pulmonary function test, volitional and non-volitional measurements of ventilatory muscles (MIP, MEP and SNIP), invasive measurements (Pes, Pga, Pdi, Sniff/Pes, Sniff/Pga and Sniff/Pdi) and analyze ventilatory accessory muscles using surface EMG. All tests were at rest and during CPET. Results: Patients with ALS (n = 28) had a preserved health status according to the results from ALSFRS-r TOTAL. Symptoms of shortness of breath measured by mMRC scales, BDI and ALSFR-s (respiratory domain) have shown none or mild symptoms compared with the control group (n=23). Patients with ALS have shown pulmonary function test results as (FEV1, FVC, SVC, and IC all in percentage of predicted.) within the range of clinical normality, although they were statically reduced compared with a control group. Inspiratory and expiratory muscle strength, measured by different methods, and especially inspiratory muscle endurance capacity are reduced and outside of normality range in patients with ALS, as well as a reduction in quality of life and level of physical activity compared to controls. The invasive method with magnetic stimulation did not find any difference between the groups at rest and does not seem to be a good measurement alternative in this population. however, the measures of ventilatory muscle strength with the volitional method were lower in the ALS group. CPET in the T-0 period showed lower work capacity, lower ventilatory efficiency, and greater recruitment of accessory muscles at with increasing effort, as a probable compensatory mechanism, with no difference in perceived exertion between the groups at peak exercise. The rate of disease progression showed lower values for capacities, functions, and perceptions of symptoms in most assessments, however without statistical significance in most cases. Conclusions: Oligosymptomatic ALS patients have impaired quality of life, daily activities and reduced ventilatory muscles strength by different methods and especially in the endurance inspiratory test, which showed a more pronounced dysfunction of the inspiratory muscles. In this group of patients, there was even greater significant disagreement between lung function and adequate measures of ventilatory force. After six months, there was disease progression with a decrease in functions, capabilities, and perception of symptoms, however without statistical difference in most of the tests performed (AU) | |
| FAPESP's process: | 14/08331-9 - Prospective evaluation of ventilatory muscle at rest and during exercise in patients with amyotrophic lateral sclerosis (ELA) and a control group |
| Grantee: | Vinicius Carlos Iamonti |
| Support Opportunities: | Scholarships in Brazil - Doctorate |