What causes als

Last updated: April 1, 2026

Quick Answer: ALS (Amyotrophic Lateral Sclerosis) is caused by progressive degeneration of motor neurons in the brain and spinal cord. The exact cause is unknown, but genetic mutations, protein misfolding, and environmental factors likely contribute to the disease.

Key Facts

Overview of ALS

Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive neurodegenerative disorder that affects motor neurons. Motor neurons are nerve cells that control voluntary muscles throughout the body. When these neurons degenerate, they can no longer send signals to muscles, causing them to weaken, atrophy, and eventually stop functioning. Most people with ALS ultimately lose the ability to move, speak, eat, and breathe on their own.

Unknown Primary Causes

Despite decades of research, the exact cause of most ALS cases remains unknown. Scientists have identified several factors that may contribute to motor neuron degeneration, but they have not determined a single cause. The disease likely results from a complex combination of genetic and environmental factors.

Genetic Factors

Approximately 5-10% of ALS cases are familial, meaning the disease runs in families. These hereditary cases result from mutations in specific genes. The most commonly mutated genes include SOD1, TARDBP, and FUS. More recently, a mutation in the C9ORF72 gene has been identified as a major cause of familial ALS, particularly in European populations. The remaining 90% of ALS cases are sporadic, occurring without a known family history, though genetic predisposition may still play a role.

Environmental and Lifestyle Factors

Researchers have investigated numerous environmental factors that may increase ALS risk:

Molecular and Cellular Mechanisms

At the cellular level, scientists believe several processes may damage motor neurons. Protein misfolding and aggregation appears to play a central role, where abnormal proteins accumulate and damage neurons. Oxidative stress and mitochondrial dysfunction also damage cells. Additionally, problems with how cells dispose of damaged proteins and components contribute to neuronal death. Inflammation in the brain and spinal cord may accelerate the degenerative process.

Risk Factors and Demographics

While anyone can develop ALS, certain groups show higher incidence rates. The disease typically develops between ages 40 and 60, though it can occur at any age. Men are slightly more likely to develop ALS than women. A family history of ALS or frontotemporal dementia significantly increases risk.

Related Questions

How is ALS diagnosed?

ALS diagnosis typically involves a combination of clinical examination, patient history, and diagnostic tests. Electromyography (EMG) and nerve conduction studies help confirm motor neuron damage by measuring electrical activity in muscles.

Is ALS hereditary?

About 5-10% of ALS cases are hereditary (familial ALS), with mutations in genes like SOD1 and C9ORF72. The remaining 90% are sporadic cases with no clear genetic link, though genetic factors may still contribute.

What is the life expectancy with ALS?

Most people with ALS live 2-5 years after diagnosis, though some survive longer. However, approximately 10% of patients survive longer than 10 years depending on the disease progression rate.

Sources

  1. National Institute of Neurological Disorders and Stroke - ALS Public Domain
  2. Wikipedia - Amyotrophic Lateral Sclerosis CC-BY-SA-4.0
  3. ALS Association - About ALS Copyright ALS Association