What Is ALS?
Amyotrophic lateral sclerosis (ALS) is
a degenerative disease that affects the brain and spinal cord. ALS is a chronic
disorder that causes a loss of control of voluntary muscles. The nerves
controlling speech, swallowing, and limb movements are often affected.
Unfortunately, a cure has not yet been found for it.
The famous baseball player Lou Gehrig
was diagnosed with the disease in 1939. ALS is also known as Lou Gehrig’s disease.
What Are the Causes of ALS?
ALS can be classified as either
sporadic or familial. Most cases are sporadic. That means no specific cause is
The Mayo Clinic estimates that genetics only cause ALS in about 5 to 10
percent of cases. Other causes of ALS aren’t
well understood. Some factors that scientists think might contribute to ALS
- free radical
- an immune
response that targets motor neurons
- imbalances in
the chemical messenger glutamate
- a buildup of abnormal proteins inside nerve cells
The Mayo Clinic has also identified smoking, lead exposure, and military
service as possible risk factors for this condition.
The onset of symptoms in ALS usually
occurs between the ages of 50 and 60, although symptoms can occur earlier. ALS
is slightly more common in men than in women.
How Often Does ALS Occur?
According to the ALS Association, every year about 6,400 people in the United States are
diagnosed with ALS. They also estimate that around 20,000 Americans are
currently living with the disorder. ALS affects people in all racial, social,
and economic groups.
This condition is also becoming more
common. This may be because the population is aging. It could also be due to
increasing levels of an environmental risk factor that hasn’t been identified yet.
What Are the Symptoms of
Both sporadic and familial ALS are
associated with a progressive loss of motor neurons. The symptoms of ALS depend
on what areas of the nervous system are affected. These will vary from person-to-person.
The medulla is the lower half
of the brainstem. It controls many of the body’s autonomic functions. These include breathing, blood
pressure, and heart rate. Damage to the medulla can cause:
lability, which is characterized by
excessive emotional reactions such as laughing or crying
- a loss of
tongue muscle contour, or tongue atrophy
- excess saliva
- difficulty breathing
The corticospinal tract is a part of the
brain made up of nerve fibers. It sends signals from your brain to your spinal
cord. ALS damages the corticospinal tract and causes spastic limb weakness.
The anterior horn is the front part
of the spinal cord. Degeneration here can cause:
- limp muscles,
or flaccid weakness
- breathing problems caused by weakness in the diaphragm and
other respiratory muscles
Early signs of ALS may include
problems performing everyday tasks. For example, you may have difficulty
climbing stairs or getting up from a chair. You might also have difficulty
speaking or swallowing, or weakness in your arms and hands. Early symptoms are
usually found in specific parts of the body. They also tend to be asymmetrical,
which means they only happen on one side.
As the disease progresses, the symptoms
generally spread to both sides of the body. Bilateral muscle weakness becomes
common. This can lead to weight loss from muscle wasting. The senses, the
urinary tract, and bowel function usually remain intact.
Does ALS Affect Thinking?
Cognitive impairment is a common
symptom of ALS. Behavioral changes can also occur without mental decline.
Emotional lability can occur in all ALS sufferers, even those without dementia.
Inattention and slowed thinking are
the most common cognitive symptoms of ALS. ALS-related dementia can also occur
if there’s cell degeneration
in the frontal lobe. ALS-related dementia is most likely to occur when there’s a family history of dementia.
How Is ALS Diagnosed?
ALS is usually diagnosed by a
neurologist. There’s no specific test
for ALS. The process of establishing a diagnosis can take anywhere from weeks
Your doctor may start to suspect you
have ALS if you have progressive neuromuscular decline. They’ll watch for increasing problems with symptoms such as:
- muscle weakness
These symptoms can also be caused by a
number of conditions. Therefore, a diagnosis requires your doctor to rule out
other health problems. This is done with a series of diagnostic tests
- an EMG to
evaluate the electrical activity of your muscles
conduction studies to test your nerve function
- an MRI that
shows which parts of your nervous system are affected
- blood tests to evaluate your general health and nutrition
Genetic tests may also be useful for people
with a family history of ALS.
How Is ALS Treated?
As ALS progresses, it becomes more
difficult to breathe and digest food. The skin and subcutaneous tissues are
also affected. Many parts of the body deteriorate, and all of them must be
Because of this, a team of doctors and
specialists often work together to treat people with ALS. Specialists involved
in the ALS team might include:
- a neurologist
who is skilled in the management of ALS
- a physiatrist
- a respiratory
- a speech therapist
- a social worker
- a mental health professional
- a pastoral care provider
Family members should talk to people
with ALS about their care. People with ALS may need support when making medical
Riluzole (Rilutek) is currently the only drug approved for the treatment of
ALS. It can prolong life by several months, but it cannot eliminate the symptoms
entirely. Other medications may be used to treat the symptoms of ALS. Some of
these medications include:
bisulphate, baclofen, and diazepam for cramping and
anti-inflammatory drugs (NSAIDs), the anticonvulsant gabapentin, tricyclic
antidepressants, and morphine for pain management
- Neurodex, which is a combination of vitamins B-1, B-6, and
B-12, for emotional lability
Scientists are investigating the use
of the following for a subtype of hereditary ALS:
- coenzyme Q10
- COX-2 inhibitors
However, they have not yet been proven
Stem cell therapy also hasn’t been proven to be an effective treatment for ALS.
What Are the Complications of ALS?
The complications of ALS include:
- bed sores
What Is the Long-Term Outlook for People with ALS?
The typical life expectancy for a
person with ALS is two to five years. Approximately 20 percent of patients live with ALS for over five years. Ten percent
of patients live with the disease for over 10 years.
The most common cause of death from
ALS is respiratory failure. There’s currently no cure for ALS. However, medication and
supportive care can improve your quality of life. Proper care can help you live
happily and comfortably for as long as possible.