In the movie ‘Tuesdays With Morrie’, Mitches old professor Morrie has been diagnosed with ALS.
What is ALS?
ALS, or amyotrophic lateral sclerosis, is a progressive disease that affects in your brain and spinal cord. This disease is always fatal. ALS usually strikes people in between the ages of 40 and 60 years old.
- Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their demise.
- When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, people may lose the ability to speak, eat, move and breathe. The motor nerves that are affected when you have ALS are the motor neurons that provide voluntary movements and muscle control.
Facts You Should Know About ALS
- ALS is not contagious.
- Riluzole, the drug approved for ALS, was approved by the FDA in late 1995. This drug was shown scientifically to prolong the life of persons with ALS by at least a few months. More recent studies show Riluzole slows the progress of ALS, allowing the patient more time in the higher functioning states when their function is less affected by ALS.
- ALS occurs throughout the world with no racial and ethnic boundaries and can affect anyone.
- Once ALS starts, it almost always progresses. It eventually takes the ability of walking, dressing, writing, speaking, swallowing and breathing. It also shortens the lifespan. The average survival rate after being diagnosed is 3 years. 20% of people who have ALS live 5 years, 10% will live 10 years and about 5% will live 20 years or more.
- Progression is not always a straight line in an individual either. It is not uncommon to have periods lasting weeks to months where there is very little or no loss of function. Less than 1% of patients with ALS will have significant improvement in function lasting 12 months or more
- Military veterans are approximately twice as likely to develop ALS.
- The onset of ALS often involves muscle weakness or stiffness as early symptoms. Progression of weakness, wasting and paralysis of the muscles of the limbs and trunk as well as those that control vital functions such as speech, swallowing and later breathing generally follows.
- With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.
- The initial symptoms of ALS vary from different people. Some people could have trouble lifting a pen or a cup of coffee, others could experience a change in pitch while speaking. ALS is typically a disease that involves a gradual onset.
- Gradual onset, painless, progressive muscle weakness is the most common initial symptom in ALS. Other early symptoms vary but can include tripping, dropping things, abnormal fatigue of the arms and/or legs, slurred speech, muscle cramps and twitches, and/or uncontrollable periods of laughing or crying.
- When the breathing muscles become affected, people with ALS will need permanent ventilator support to assist with breathing.
- Since ALS attacks only motor neurons, the sense of sight, touch, hearing, taste and smell are not affected.
- ALS is a difficult disease to diagnose. There is no one procedure that diagnosis this disease. You must undergo a series of clinical examinations to rule out the possibility of diseases that mimic ALS. A diagnostic workup includes most, if not all, the following procedures:
- Blood and urine studies
- Spinal tap
- X-rays, including magnetic resonance imaging (MRI)
- Myelogram of cervical spine
- Muscle and/or nerve biopsy
- A thorough neurological examination
Forms of ALS
There are two main classifications of ALS:
- Sporadic – the most common form of ALS – 90 to 95% of all cases.
- Familial – occurring more than once in a family lineage (genetic dominant inheritance) – 5 to 10% of all cases.
The most common form of ALS is “sporadic” ALS. It may affect anyone, anywhere. “Familial” ALS means the disease is inherited. Only about 5 to 10% of all ALS patients appear to have genetic or inherited form of ALS. In those families, there is a 50% chance each offspring will inherit the gene mutation and may develop the disease.