PROJECT revELA: MONITORING SYMPTOMS OF INDIVIDUALS WITH
AMYOTROPHIC LATERAL SCLEROSIS - A LONGITUDINAL ONE-YEAR
STUDY
Amyotrophic Lateral Sclerosis; Motor neurons; longitudinal study
Introduction: Amyotrophic Lateral Sclerosis (ALS) is a progressive, ultimately
fatal, neurodegenerative disease. The degeneration occurs in motor neurons
located in the cerebral cortex and spinal cord, leading to muscle weakness
and atrophy. Due to the heterogeneity of the disease, its clinical presentation
is varied, being classified as spinal ALS – when there is greater involvement
in the limbs – and bulbar ALS, when it begins with bulbar symptoms.
Objective: To monitor the evolution, over a one-year period, of motor and
non-motor symptoms in individuals with ALS. Methods: This is a longitudinal,
analytical, and prospective study. The recruited participants had a diagnosis
of ALS, of both sexes, over 18 years of age, followed by the reference
hospital in the treatment of people with ALS. The instruments ALSFRS-r
(Revised Amyotrophic Lateral Sclerosis Functional Rating Scale) were
applied to assess functionality; EVA (Visual Analogue Scale) and body
diagram for pain assessment; ALS-CBS (Cognitive Behavioral Screen) for
cognition assessment; ESF (Fatigue Severity Scale) for fatigue assessment;
in addition to an assessment form containing clinical and sociodemographic
data. The instruments were applied in person or remotely every three months,
totaling five evaluations. For data analysis, the Statistical Package for Social
Sciences (SPSS) version 25.0 and JASP version 0.18.3.0 software were
used. Longitudinal comparisons of outcome variables were analyzed via
Generalized Estimating Equations with Tukey's post hoc. Bivariate
association analyzes were performed via Pearson's correlation test, when the
correlations were between continuous variables; point-biserial correlation, between continuous and categorical variables; and the Chi-square test
between categorical variables. To assess the predictors of functionality,
Marginal Regression Models were used. Results: The sample consisted of
70 participants, 41 males and 29 females, with a mean age of 57 years. 66%
of individuals had a confirmed diagnosis of ALS, with 83% being of the spinal
type and 17% of the bulbar type. There was no statistical difference between
the five assessment moments of functionality, pain, fatigue, and cognition. In
the marginal model for predicting functionality, the use of an auxiliary device
for locomotion reduced the score on the functional scale by 6.8 points. The
presence of pain decreased functionality by 3.1 points as seen on the
ALSFRS-R scale. Older age reduced the functional score by 0.2 points. For
cognition, higher scores on the ALS-CBS scale increased the score on the
functional scale by 0.9 points. Higher levels of fatigue indicated a reduction of
0.7 points in functionality. Conclusion: This study showed that there appears
to be no reduction in functionality over a one-year period in a population
followed by a multidisciplinary team. Furthermore, motor and non-motor
variables may have an influence on functionality in distinct ways.