Lou Gehrig’s disease, known as amyotrophic lateral sclerosis or ALS, strikes healthy, middle-aged people seemingly at random, and, of the major neurodegenerative diseases, it has the least hope for treatment and survival. Although mental capabilities stay intact, ALS paralyzes people, often from the outside in, and most patients die within three years, when they can no longer breathe or swallow. At any given time, an estimated 30,000 are fighting for their life with it in this country. We each have about a 1 in 400 chance of developing this dreaded disease.
ALS is a progressive disease that attacks nerve cells (neurons) in the spinal cord and brain, which dictate the actions of voluntary muscles. When the lower and upper motor neurons deteriorate the muscles they influence slowly break down and waste away, resulting in paralysis.
ALS is a fatal degenerative disorder of upper and lower motor neurons that affects muscle functions, including breathing, speaking and swallowing. In about 50% of cases, ALS also is associated with cognitive impairment ranging from frontotemporal dementia to milder forms of executive or dysexecutive impairment. In most cases ALS appears sporadically in the population; only about 10% of patients have a positive family history for ALS or frontotemporal dementia.
Most people who develop ALS are between 40 and 70 years old and are usually diagnosed around the age of 55. More than 50 percent of all ALS patients live more than three years after being diagnosed, even though the average life expectancy of a person with ALS is between two and five years.
The speed and order of the disease’s progression varies from person to person.
The brain and spinal cord are called the
central nervous system.
The nerves in the body such as the arms
legs chest abdomen and pelvis make up
the peripheral nervous system.
The main cells that make up the brain
and the spinal cord are called neurons.
The neurons are the thinking cells of
the brain they communicate with each
other by sending electric signals.
Through wires called axons neurons that
control our muscles are called motor
neurons.
ALS attacks the neurons that control
muscles. The motor neurons messages from
motor neurons in the brain called upper
motor neurons are transmitted to motor
neurons in the spinal cord called lower
motor neurons then passed on to the
muscles.
With ALS both the upper motor neurons
and the lower motor neurons die and stop
sending messages to the muscles.
Symptoms of ALS usually occur very
gradually at first they may be mistaken
for symptoms of other less serious
diseases.
Symptoms of ALS are caused by destroyed
motor neurons the muscles affected by
the destroyed motor neurons gradually
weaken waste away and twitch.
Early symptoms of ALS affect the parts
of the body where the motor neurons got
damaged first.
In some cases ALS affects only one leg
at first patients notice that they are
awkward when they walk or run.
Some ALS patients notice the first signs
of ALS in their hand or arm they may
find that simple tasks such as buttoning
a shirt writing or turning a key in a
lock or difficult other patients notice
speech problems.
ALS causes a wide range of disabilities
eventually the brain's ability to
control voluntary movement is lost.
Patients lose strength and the ability
to move their arms legs and body.
When muscles in the diaphragm and chest
wall no longer work, ALS patients cannot
breathe without support from a machine.
Most people with ALS experience major
respiratory problems within three to
five years of the first symptoms.
Since ALS only affects motor neurons the
disease does not impair personality
intelligence or memory.
ALS does not affect the ability to see
smell taste here or recognize touch ALS
patients usually retain control of eye
muscles and bladder and bowel functions.
ALS is usually difficult to diagnose
early because it can appear to be
another disease such as spinal cord
tumors multiple sclerosis or nerve
compression.
As the symptoms of ALS progress it
becomes easier to diagnose.
ALS can be diagnosed using electric
tests on the muscles and nerves these
tests are called EMG and NCV for
electromyogram and nerve conduction
velocity.
It's been known for quite some time that there are variants of ALS. One is referred to as progressive muscular atrophy, or PMA. It appears to be an isolated illness of the lower motor neurons. However, pathologically, if you do an autopsy of a patient, they will have evidence of deterioration of upper motor neurons.
There is also primary lateral sclerosis and clinically it looks like an isolated upper motor-neuron disorder. However, pathologically they also have lower motor-neuron disorder.
The other classic syndrome is called progressive baldor palsy or progressive supranuclear palsy which is weakening of cranial muscles, like the tongue, face and swallowing muscles. But it pretty much always spreads to limb muscles.
Those are the four classic motor-neuron disorders that have been described. And it was thought for quite some time that these disorders were limited to motor neurons. It's now clear that that's not true. It's now well recognized that 10 percent of these patients can develop degeneration in another part of the brain, such as other parts of the frontal lobe that don't contain the motor neurons or the temporal lobe. So some of these patients can actually develop dementia, called frontal-temporal lobe dementia.
One of the misconceptions about ALS is that it's only a motor-neuron disease, and that's not true.
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