From the Guidelines
The primary cause of death in patients with neurodegenerative diseases such as Multiple Sclerosis (MS) and Amyotrophic Lateral Sclerosis (ALS) is respiratory failure and malnutrition with dehydration.
Key Factors Contributing to Mortality
- Respiratory failure: Due to the progressive nature of ALS, patients often experience respiratory muscle weakness, leading to respiratory failure 1.
- Malnutrition and dehydration: Malnutrition is a common issue in ALS patients, resulting from factors such as dysphagia, dysarthria, and decreased mobility, which can lead to dehydration and further exacerbate the condition 1.
Nutritional Status and Prognosis
- Weight loss: Significant weight loss (>10%) is associated with increased risk of death in ALS patients 1.
- Body mass index (BMI): Lower BMI at diagnosis is associated with shorter survival, while weight gain is associated with improved survival 1.
- Bioelectrical impedance phase angle (PA): Higher PA is associated with reduced risk of death, while a decrease in PA is associated with increased risk of death 1.
Gastrostomy Feeding
- Gastrostomy feeding: Recommended for patients with severe dysphagia to provide long-term enteral nutrition therapy, which may improve survival and quality of life 1.
- Timing of gastrostomy insertion: Early gastrostomy may be beneficial, as it can prevent significant weight loss and improve nutritional outcomes 1.
From the Research
Causes of Death in Neurodegenerative Diseases
The causes of death in patients with neurodegenerative diseases such as Multiple Sclerosis (MS) and Amyotrophic Lateral Sclerosis (ALS) can be attributed to various factors, including:
- Cell death and apoptosis, which are common features of neurodegenerative diseases 2
- Degenerative and inflammatory processes, which can lead to a cascade of production of reactive oxygen species and nitric oxide, resulting in cell death and apoptosis 3
- Neuroinflammation and neurodegeneration, which are intricately related in these diseases 4
Pathological Features
The pathological features of concurrent MS and ALS have been examined in several studies, which have shown:
- Characteristic "old" plaques of multiple sclerosis in the cerebrum with anterior horn cell and pyramidal tracts degeneration, typical of amyotrophic lateral sclerosis, in the spinal cord 5
- "Burned out" plaques of multiple sclerosis and anterior horn cell and axonal degeneration of amyotrophic lateral sclerosis 6
- TDP-43 inclusions, which are a hallmark of ALS, in patients with concurrent MS and ALS 4
Mechanisms of Cell Death
The mechanisms of cell death in MS and ALS are complex and multifactorial, involving:
- Apoptosis, which is a programmed cell death pathway that is dependent on the cell's internal metabolism, environment, developmental background, and genetic information 2
- Necrosis, which is a result of cellular "accidents" such as those occurring in tissues subjected to chemical trauma 2
- Reactive oxygen species and nitric oxide, which can lead to cell death and apoptosis 3