Amyotrophic Lateral Sclerosis: Exploring Disability Beyond Motor Weakness
摘要
Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disorder characterized by the loss of upper and lower motor neurons, resulting in debilitating muscle weakness. This leads to difficulties with activities of daily life, speech impairment, and ultimately culminates in respiratory failure and death. Additionally, ALS causes a spectrum of nonmotor symptoms through both direct (related to the involvement of nonmotor neurons) and indirect mechanisms (secondary to weakness), including neuropsychiatric symptoms, pain, fatigue, and sleep disturbances. Thus, ALS exemplifies the breadth of disability observed in neurological disorders, which, over the last few decades, have emerged as the foremost cause of global ill health and disability. Similar to other neurological disorders lacking curative interventions, treatment options for ALS are limited to symptomatic management, emphasizing the crucial need for their identification and acknowledgment. Beginning with the evaluation of motor disability in ALS, with a specific focus on the unique challenge posed by speech impairment, this entry then explores additional, nonmotor, sources of disability. The goal of this entry is to highlight the diverse and often underdiagnosed symptoms of ALS, emphasizing their profound impact on patient survival, quality of life, and the substantial burden they place on caregivers.