Cecilia Quarracino1, Natalia Bohorquez Morera2, Francisco Capani1, Santiago Pérez-Lloret3, Gabriel Eduardo Rodríguez2
1Laboratorio de citoarquitectura y plasticidad neuronal, CAECIHS UAI-CONICET, 2Motor Neuron Disease Clinic, Neurology Department, Hospital de agudos José María Ramos Mejía, 3Observatorio de Salud Pública, Pontificia Universidad Católica Argentina, Consejo de Investigaciones Científicas y Técnicas (UCA-CONICET)
Objective:
Study the presence of cardiac dysautonomia in ALS patients.
Background:
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting the upper and lower motor neurons. Diffuse neuronal affection has been observed in more frequent neurological diseases of degenerative etiology. There are isolated and sometimes contradictory studies addressing cardiac dysautonomia in ALS. Both orthostatic hypotension (sympathetic nervous system) and reduced RR interval variability (parasympathetic nervous system) are associated with a higher morbimortality.
Design/Methods:
Demographic variables, ALS-FRS and MRC scores and forced vital capacity (FVC) were registered in 20 ALS patients (with definite or probable diagnosis according to El Escorial Revisited criteria) attending a regular neurological control. We performed dynamometry, orthostatic testing and manually evaluated the RR interval variability in resting, deep breathing and post Valsalva maneuver electrocardiograms. All patients signed the informed consent approved by the hospital’s ethics committee. Logistic regression was used to address the relationship between cardiac parasympathetic dysautonomia and the independent relevant variables.
Results:
The mean age was 52 years (SD 13.79), with 75% of male patients and 85% of spinal onset. The mean ALS-FRS and FVC scores were 25.65 (SD 10.55) and 67% (SD 21). Only one patient had orthostatic hypotension while 12 (58.8%) showed reduced RR interval variability, with a strong tendency of association with age and not any other variable.
Conclusions:
Parasympathetic cardiac dysautonomia was very frequent in ALS patients. Larger studies are needed to confirm this finding since its diagnosis could help to tailor treatment and follow up in order to reduce other possible causes of morbimortality in ALS patients.