Cardiac autonomic findings in amiotrophic lateral sclerosis

Introduction and Purpose: Amyotrophic lateral sclerosis (ALS) is a progressive and lethal disease characterized by degeneration of the corticospinal tract, brain stem and spinal cord in the upper and lower motor neurons. Although it is thought that ALS does not affect the autonomic nervous system, there is growing evidence that the autonomic nervous system is affected by this disease as part of a degenerative process. In our study, our aim was to determine the demographic features of patients with definite Amyotrophic Lateral Sclerosis, examine the environmental risk factors, and to show the presence of cardiac autonomic involvement. Materials and Methods: This prospective study was carried out in 61 patients with definite Amyotrophic Lateral Sclerosis in Neuromuscular Diseases Policlinic of Neurology Department at Cukurova University Faculty of Medicine between September 2016 and January 2018. Participants included similar age groups and 29 control groups with similar gender distribution. Demographic data (age, gender, age at onset of symptom, place of birth and place of residence), past medical history (smoking, head trauma, spinal injury, spinal surgery, psychiatric treatment, comorbidity) and family history (amyotrophic lateral sclerosis patient, other neurodegenerative diseases and cancer presence) were questioned. The initial localization of the disease was questioned and disease disability was assessed with ALSFDS. Patients were evaluated for the presence of orthostatic hypotension for cardiac autonomic involvement. Transthoracic echocardiography and holter-ECG were performed on the patient and control group. Results: The mean age at onset of ALS was found to be 56.6 and was 1.4 times more frequent in males than females. It was determined that the disease started in men earlier than women. Most of the patients were sporadic and only four index cases were diagnosed as familial ALS. Two patients were diagnosed with ALS-Dementia. Two other patients were diagnosed with ALS-Parkinsonism complex. It was determined that neurodegenerative disease and cancer history were very common in the family. Orthostatic hypotension was detected in 21.3% of the patients. There was no significant difference in the echocardiography findings between the patient and control groups in terms of systolic dysfunction, diastolic dysfunction, left ventricular hypertrophy, and valve pathologies. In Holter electrocardiography examinations, no significant difference was found between the patient and the control groups when the heart rate variability time domain evaluation parameters were evaluated. The low frequency and very low frequency parameters of the frequency domain evaluation parameters were significantly lower in the patient group than the control group. Conclusion: In ALS cases, cardiac autonomic dysfunction occurs in every phase of the disease. In order to fully understand the pathophysiology of autonomic dysfunction, there is a need for larger studies to determine the relationship between motor neuron degeneration and autonomic neuron degeneration.