Amyotrophic Lateral Sclerosis

What is amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis, known also as ALS or Lou Gehrig’s disease, is a nervous system disease. ALS affects the nerve cells in the brain and in the spinal cord. Patients who receive an amyotrophic lateral sclerosis diagnosis will lose muscle control as the disorder progresses. The precise cause of ALS remains unknown and there are only a small number of inherited cases.

Although there is no cure for the fatal disease, amyotrophic lateral sclerosis symptoms can be managed. Speak immediately to a healthcare provider once you notice possible symptoms.

What are symptoms of amyotrophic lateral sclerosis?

Back to top

Each person’s ALS symptoms differ and relate to the affected nerve cells. You or a loved one might notice muscle weakness that spreads and worsens with time. Other symptoms are:

  • Tripping and falling
  • Weakness (legs, feet, or ankles)
  • Slurred speech
  • Trouble swallowing
  • Difficulty walking or doing common activities
  • Sudden crying, laughing, or yawning
  • Hand weakness or clumsiness
  • Weakness due to muscle cramps
  • Twitching in the arms, shoulders, or tongue
  • Thinking and/or behavioral changes

 

ALS typically shows early symptoms in the hands, feet, arms, or legs. It spreads to other areas of the body as the disorder progresses and the muscles get weaker. Later stage symptoms will include difficulty with:

  • Chewing food and swallowing
  • Speaking or forming words
  • Drooling
  • Breathing
  • Making bowel movements
  • Maintaining a healthy weight

 

It is unlikely for patients to experience pain in the early stages of amyotrophic lateral sclerosis. Patients also rarely feel pain in later stages of disease progression. ALS rarely affects:

  • Bladder control
  • The senses (taste, smell, touch, hearing)

 

If you notice these or any other unexplained, sudden changes, consult your physician or make an appointment with a specialist.

What causes amyotrophic lateral sclerosis?

Back to top

A genetic cause can be identified in about 10% of people who have ALS. The cause of the disease remains unknown in all other patients. Researchers theorize a complex interaction between genes and environmental factors plays a role in ALS.

How is amyotrophic lateral sclerosis diagnosed?

Back to top

Getting an accurate ALS diagnosis early can allow for more effective treatment soon after the start of any symptoms. Scheduling an appointment with a neurologist who is familiar with ALS can effectively lead to an early diagnosis. While no singular test can definitively diagnose ALS, doctors will:

  • Conduct a physical examination
  • Review a patient’s full medical history

 

Neurologic examinations should be performed at regular intervals. These exams will determine if your symptoms are advancing with time. Neurologic exams allow a doctor to test your:

  • Reflexes
  • Muscle strength
  • Other responses

 

You will undergo muscle and imaging tests to rule out other disorders, which will allow a doctor to diagnose the patient with ALS. Tests can include a magnetic resonance imaging (MRI) or an electromyography (EMG). An EMG used to diagnose amyotrophic lateral sclerosis can include:

  • A nerve conduction study (NCS): Measuring the electrical activity of nerves and muscles
  • A needle exam: Detecting electrical activity in the muscle fibers

 

Doctors may rule out other possible disorders with tests, such as:

  • Muscle biopsy
  • A spinal tap
  • Blood and urine tests

 

Setting up a consultation with a doctor is better sooner rather than later. Talking to a healthcare provider will help them to determine which diagnostic method might yield the quickest and most accurate results.

What are the risk factors for amyotrophic lateral sclerosis?

Back to top

There are certain established risk factors for ALS. These are:

  • Genetics: A risk gene is passed down from a family member in about 10% of people with ALS. The kids of individuals with hereditary amyotrophic lateral sclerosis have a 50% of inheriting the gene.
  • Age: ALS is most common for individuals between the ages of 60 and mid-80’s. The risk of an amyotrophic lateral sclerosis diagnosis increases with age, up to the age of 75.
  • Sex: Slightly more men than women develop ALS before the age of 65. There is less of a discrepancy between the sexes after the age of 70.

 

Certain environmental factors can contribute to an increased risk of ALS. These are:

  • Smoking. Women who smoke, especially after menopause, may carry a higher risk.
  • Toxin exposure. No singular agent or chemical is definitively linked with ALS, but some studies suggest that lead exposure or other workplace substances could be linked to the fatal diagnosis.

Military service. It is still unclear what aspects of military service could trigger ALS. There is some thought that exposure to metals and chemicals or experiencing viral infections, intense exertion, or traumatic injuries could trigger the disease.

Does amyotrophic lateral sclerosis cause complications?

Back to top

Different complications will arise as ALS progresses.

  • Breathing difficulty. ALS patients may require the use of a mask ventilator to help them breathe at night. Advanced cases of amyotrophic lateral sclerosis might call for a patient to undergo a tracheotomy, or a surgically created hole at the front of the neck. Breathing failure is the most common cause of death for individuals with ALS.
  • Speaking problems. As slower and occasionally slurred speech progresses to difficulty understanding speech, other forms of communication and technology are often used to communicate with people who have ALS.
  • Eating problems. A feeding tube can help to reduce the risk of food, liquids, or saliva entering into the lungs, resulting in pneumonia or aspirating. Using a feeding tube can also provide proper hydration and nutrition in patients.
  • Dementia. Difficulty with language and decision-making can lead to people with ALS being diagnosed with frontotemporal dementia.

How is amyotrophic lateral sclerosis treated?

Back to top

No current treatment exists that can reverse the damage to motor neurons or cure ALS. Some treatments may slow progression of amyotrophic lateral sclerosis. Early treatment can improve a person’s quality of life and extend their survival.

Supportive healthcare can be found with integrated, multi-disciplinary teams. A team providing an individualized treatment plan with special equipment can often keep people who have ALS mobile, comfortable, and more independent. These teams consist of:

  • Physicians
  • Social workers
  • Pharmacists
  • Nutritionists
  • Physical, occupational, speech, and respiratory therapists
  • Clinical psychologists
  • Home care and hospice nurses

 

Certain FDA-approved medicines for the treatment of ALS may be recommended. These are:

  • Riluzole (Rilutek): An oral medicine believed to reduce damage to the motor neurons. Clinical trials suggest that the drug may extend survival for a few months in people with ALS. There is also a thickened liquid form or a dissolving tablet for people who struggle to swallow pills.
  • Edaravone (Radicava): An oral or intravenous antioxidant with proven ability of slowing the functional decline of some people with amyotrophic lateral sclerosis.
  • Tofersen (Qalsody): Delivered by a spinal injection in ALS patients with a confirmed mutation in the SOD1 gene. The benefits of the drug are still being studied.

 

There are other medicines or treatments that a doctor may prescribe to help manage symptoms. Certain prescriptions can help to control:

  • Muscle cramps and stiffness
  • Excessive saliva and phlegm
  • Unwanted emotional displays
  • Pain
  • Depression
  • Sleep problems
  • Constipation

 

Rehabilitation in the form of physical, occupational, and speech therapy are common for people with amyotrophic lateral sclerosis. Active physical treatment can help maintain muscle strength and function while helping the individual keep as much independence as possible for longer.

A doctor can outline the best combination of palliative and lifestyle treatments that make sense for your specialized care.

What is the outlook for amyotrophic lateral sclerosis?

Back to top

Roughly 50% of people with ALS die within 14 to 18 months of the diagnosis. Some individuals with amyotrophic lateral sclerosis live for 10 years or longer. Early ALS detection and diagnosis make a significant difference in an individual’s months or years spent living with the disease. A proactive approach is the best way forward for a heightened quality of life.

Categories

Archives