Amyotrophic lateral sclerosis. Its natural history

Area of Science:

• Neurology
• Motor Neuron Diseases

Background:

• Amyotrophic lateral sclerosis (ALS) is the most prevalent motor neuron disease (MND).
• Other MNDs include progressive bulbar palsy (PBP), progressive muscular atrophy (PMA), and primary lateral sclerosis (PLS).

Purpose of the Study:

• To describe the clinical characteristics, patterns of progression, and survival rates in patients with ALS.
• To analyze the relationship between the area of symptom onset and disease progression.

Main Methods:

• Retrospective analysis of patient data including age of onset, sex, initial symptoms, and progression patterns.
• Assessment of symptom involvement, muscle function, and survival duration.

Main Results:

• ALS typically begins at age 57, with a 1.5:1 male-to-female ratio.
• Common initial symptoms include lower extremity weakness (36%), upper extremity weakness (32%), and bulbar onset (25%).
• Over 90% experience muscle atrophy and fasciculations; 47% develop spasticity. A plateau in disease progression was observed in 25% of patients.

Conclusions:

• ALS presents with diverse initial symptoms and progression patterns, influenced by the onset location.
• While most patients show linear deterioration, some experience disease stabilization.
• The average survival for all MND forms in this study was 4.08 years.