Overview
Amyotrophic lateral
sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive
and fatal disease of the nervous system. ALS affects both upper motor neurons,
found in the motor cortex of the brain, and lower motor neurons, found in the
brain stem and spinal cord. This disease destroys a person’s nerve cells
and causes generalized weakness leading to paralysis and death.
Estimates indicate that four to six people per 100,000 are living with ALS at any given time. Additionally, an estimated 6000 people in the United States are diagnosed with ALS each year, with a prevalence of nearly 20,000.
Causes
Researchers still don’t know
exactly what causes motor neurons to die with ALS. Several possible
causes that researchers are studying include:
- Gene mutation
Various genetic mutations can lead to inherited ALS. C9orf72 gene, SOD1 gene,
TARDBP and FUS gene mutations account for a relatively large proportion of familial
ALS
- Chemical imbalance
Excessively high levels of glutamate will poison some nerve cells in the
spinal fluid.
- Disorganized immune response
In an abnormal state, a person’s immune system might attack and kill his
or her body’s normal cells.
- Protein mishandling
Mishandled proteins within the nerve cells may lead to a gradual accumulation of abnormal forms of these proteins in the cells.
Symptoms
Early signs and
symptoms of ALS include:
- Difficulty walking or doing your normal
daily activities - Difficulty expressing emotions and doing
cognitive activities - Tripping and falling
- Weakness in your leg, feet or ankles
- Hand weakness, clumsiness or stiffness
- Slurred speech or trouble swallowing
- hoarseness
- Poor speech volume
- Muscle cramps and twitching
- Difficulty holding your head up or
keeping good posture
As the disease advances, you will experience the flowing symptoms:
- Progressive weakening of muscles
- Paralysis of the whole body
- difficulty in speaking, swallowing, and eventually, breathing.
Diagnosis
It can be a challenge to diagnose Amyotrophic Lateral Sclerosis early,
because
it shares some similar symptoms with many other neurological
diseases. Your doctor will diagnose ALS by tracking the progression of the
symptoms and conducting several clinical tests, including:
- Electromyogram (EMG)
- Nerve conduction study
- Magnetic resonance imaging (MRI)
- Blood and urine tests
- Spinal tap (lumbar puncture)
- Muscle biopsy
Treatment
There doesn’t exist any treatment to cure amyotrophic lateral
sclerosis permanently until now. However, the following treatments may have a
positive effect, such as slowing the progression of symptoms, preventing
complications and improving the quality of your life.
Medications
Two
medications have been proven helpful in managing the symptoms of ALS:
Your doctor may also prescribe medications
to provide relief from other symptoms, including:
- Muscle
cramps and spasms - Spasticity
- Constipation
- Fatigue
- Excessive
salivation - Excessive
phlegm - Pain
- Depression
- Sleep
problems - Uncontrolled
outbursts of laughing or crying
Therapies
Some of the useful therapies include:
- Breathing care
- Physical therapy and exercise
- Occupational therapy
- Speech therapy
- Nutritional support
- Psychological and social support.
- Hot tub and whirlpool baths
- Dietary counseling
Keywords: amyotrophic lateral sclerosis, ALS.
