Multimodal assessment of autonomic dysfunction in amyotrophic lateral sclerosis

TY - JOUR

T1 - Multimodal assessment of autonomic dysfunction in amyotrophic lateral sclerosis

AU - Weise, David

AU - Menze, Ina

AU - Metelmann, Moritz C. F.

AU - Woost, Timo B.

AU - Classen, Joseph

AU - Pelz, Johann Otto

PY - 2021

Y1 - 2021

N2 - Background and purpose: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder with predominant progressive degeneration of motor neurons and motor deficits, but non-motor symptoms (NMS) such as cognitive and behavioural deficits are frequent and underestimated in current diagnostic pathways. Autonomic dysfunction has occasionally been described, although its frequency and relevance are unclear. The aim of this study was to investigate the role of the autonomic nervous system in ALS using a multimodal approach.Methods: Thirty-seven ALS patients and 40 healthy sex- and age-matched controls were included. NMS were studied with the NMS assessment scale for Parkinson's disease and an autonomic subscale was calculated. Cardioautonomic innervation at rest and whilst standing was assessed by different parameters of heart rate variability. Morphological changes (cross-sectional area) of the vagus and median nerves for control were measured with high-resolution ultrasound.Results: Non-motor symptoms in general were more frequent in ALS patients and correlated inversely with the ALS Functional Rating Scale whereas the autonomic subscore of the NMS assessment scale for Parkinson's disease did not differ between the two groups and was not related to functional impairment. Cardioautonomic assessment solely revealed an increased heart rate at rest in ALS patients, whereas the other heart rate variability parameters did not differ from controls. Structural sonographic investigation of the vagus and median nerves was similar in both groups.Conclusions: Using a multimodal approach evidence was found for a rather mild cardio- sympathetic overactivity in ALS patients. Overall, autonomic dysfunction seems to be subtle and is not related to the functional state of ALS patients.

AB - Background and purpose: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder with predominant progressive degeneration of motor neurons and motor deficits, but non-motor symptoms (NMS) such as cognitive and behavioural deficits are frequent and underestimated in current diagnostic pathways. Autonomic dysfunction has occasionally been described, although its frequency and relevance are unclear. The aim of this study was to investigate the role of the autonomic nervous system in ALS using a multimodal approach.Methods: Thirty-seven ALS patients and 40 healthy sex- and age-matched controls were included. NMS were studied with the NMS assessment scale for Parkinson's disease and an autonomic subscale was calculated. Cardioautonomic innervation at rest and whilst standing was assessed by different parameters of heart rate variability. Morphological changes (cross-sectional area) of the vagus and median nerves for control were measured with high-resolution ultrasound.Results: Non-motor symptoms in general were more frequent in ALS patients and correlated inversely with the ALS Functional Rating Scale whereas the autonomic subscore of the NMS assessment scale for Parkinson's disease did not differ between the two groups and was not related to functional impairment. Cardioautonomic assessment solely revealed an increased heart rate at rest in ALS patients, whereas the other heart rate variability parameters did not differ from controls. Structural sonographic investigation of the vagus and median nerves was similar in both groups.Conclusions: Using a multimodal approach evidence was found for a rather mild cardio- sympathetic overactivity in ALS patients. Overall, autonomic dysfunction seems to be subtle and is not related to the functional state of ALS patients.

U2 - 10.1111/ene.15177

DO - 10.1111/ene.15177

M3 - SCORING: Journal article

VL - 29

SP - 715

EP - 723

JO - EUR J NEUROL

JF - EUR J NEUROL

SN - 1351-5101

IS - 3

ER -