Non-motor symptoms in amyotrophic lateral sclerosis / 西安交通大学学报(医学版)

ABSTRACT

Amyotrophic lateral sclerosis (ALS) is a degenerative disease of the nervous system caused by the loss of motor neurons in the brain and spinal cord. At present, ALS is considered as a multi-system disease, with both motor and non-motor features. Motor neuron involvement usually begins in a single functional region (medulla oblongata, cervical vertebra, lumbosacral and thoracic vertebra) and gradually spreads throughout the motor system. Non-motor features may be caused by pathological damage from the motor region to the adjacent non-motor region or by other unknown causes. There is no uniform definition of non-motor features of ALS. Sometimes they refer to symptoms caused by damage to the non-pyramidal system, and sometimes they also include ALS syndrome. They can be divided into neuropsychiatric, autonomic, vascular and gastrointestinal dysfunctions. More than 50% of patients with ALS have symptoms of neuropsychiatric disorder, which are extremely heterogeneous. Such clinical heterogeneity is based on neuroimaging, genetics and neuropathology, which has updated the concept of ALS, leading to the present recognition of the spectrum disorder of ALS and frontotemporal dementia. The core features of the ALS diagnosis model should also be multi-axis diagnosis, using three main "diagnostic axes", i.e., axis to define motor neuron symptom variations, axis Ⅱ to define cognitive and behavioral dysfunction, and axis III to define other non-motor symptoms. This paper introduces ALS cognitive and behavioral dysfunction, ALS plus syndrome and other non-motor features associated with ALS so as to improve clinicians' understanding of managing the overall health status of ALS patients, make rational scientific evidence-based decisions and contribute to improving the quality of life of ALS patients.