[Survival in amyotrophic lateral sclerosis]

Area of Science:

• Neuroscience
• Neurology
• Epidemiology

Context:

• Amyotrophic Lateral Sclerosis (ALS) is a fatal, progressive neurodegenerative disease with a median survival of approximately 3.5 years.
• Identifying prognostic factors is crucial for understanding disease progression and patient outcomes in ALS.
• This study investigates survival duration and probability in a defined ALS population.

Purpose:

• To determine the mean disease duration and survival probability in a Belgrade-based ALS cohort.
• To analyze survival differences based on patient demographics (sex, age) and clinical presentation (bulbar vs. spinal onset).

Summary:

• A 7-year survey identified 58 ALS cases; 54 were followed. Mean survival was 27.7 months. Two-, five-, and seven-year survival rates were 62%, 27%, and 27% respectively.
• Patients under 49 years and those with spinal ALS onset exhibited significantly higher survival rates compared to older patients and those with bulbar onset.
• Bulbar onset ALS showed a statistically significant poorer prognosis (no survivors at 4 years) compared to spinal onset ALS.

Impact:

• Findings indicate that advanced age and bulbar onset are associated with poorer prognosis in ALS.
• Younger patients (<49 years) demonstrate significantly better survival rates, aligning with other research.
• This study provides valuable epidemiological data on ALS prognosis, supporting the need for targeted research into factors influencing disease progression, such as the apolipoprotein Eepsilon 4 allele.