How Do People Get ALS?

Amyotrophic lateral sclerosis (ALS, also called Lou Gehrig's disease) is a rare neurological motor neuron disease that damages the nerves that control muscles, causing muscles to weaken. Over time, the muscles gradually weaken, start to twitch (fasciculations), and waste away (atrophy) and the brain loses the ability to control voluntary movements.

About 5 to 10 percent of cases of amyotrophic lateral sclerosis (ALS) occur in people who have a family history of ALS, and in those cases, the cause is believed to be genetic. 

The other 90 to 95 percent of ALS occurs sporadically, without a family history. It is believed there are genetic variations that can make a person more susceptible to sporadic ALS.

Risk factors for developing sporadic ALS include:

• Race/ethnicity: Caucasians have higher rates of ALS than other races
• Gender: prior to the age of 65-70, the incidence of ALS is higher in men than in women, but after that the incidence is equal

Possible causes of sporadic ALS include: 

• Oxidative stress
• Mitochondrial dysfunction
• Immune system abnormalities 
• Glutamate toxicity
• Toxic exposures
• The incidents of ALS cases among Gulf War veterans is two times higher than expected, possible due to cyanobacteria that live in desert sands 
• Higher-than-average rates of ALS on the island of Guam may be due to native peoples' diet of poisonous nuts from indigenous cycad trees

Tests used to help diagnose amyotrophic lateral sclerosis (ALS) include: 

• Electromyography (EMG), to check how well nerves and muscles are working
• Magnetic resonance imaging (MRI) of the brain and spinal cord
• Blood and urine tests
• Tests to rule out infectious diseases such as:
  • Human immunodeficiency virus (HIV)
  • Human T-cell leukemia virus (HTLV)
  • Polio
  • West Nile virus 

Tests to rule out neurological disorders such as:

• Multiple sclerosis
• Post-polio syndrome
• Multifocal motor neuropathy
• Spinal and bulbar muscular atrophy (Kennedy’s disease) 

There is no cure for amyotrophic lateral sclerosis (ALS), but symptoms can be treated.

Medications used to treat ALS include: 

• Riluzole (Rilutek), which works by slowing down the progression of the disease a little bit which can help patients live a few months longer
• Edaravone (Radicava) may help slow the progression of ALS in some patients

Other treatments used to treat ALS include are aimed at helping people cope with the symptoms of ALS include:

• Breathing support 
• Noninvasive ventilation (NIV) such as a mask over the face or nose
• Techniques to help increase forceful coughing, including mechanical cough assist devices and breath stacking
• Mechanical ventilation (respirators) 
• Nutritional support from a nutritionist/dietitian
• Feeding tube 

Devices and therapies to help with walking and other tasks such as:

• Cane
• Crutches
• Motorized wheelchair
• Devices to help with talking, such as a special computer that patients control with hand or eye movements 
• Physical therapy 
• Speech therapy

Medicines to treat symptoms of ALS including:

• Muscle spasms and weakness
• Drooling
• Sleep problems
• Pain
• Depression

Most people with ALS die within 3 to 5 years of symptom onset from respiratory failure but about 10% of patients survive for 10 or more years.