A.L.S (Amyotrophic Lateral Sclerosis) is the most severe of degenerative diseases that affects nerve cells. The disease is rare and incurable. The disease is also known as "Lou Gehrig's Disease," for the American Baseball player who contracted the disease in the 1930s. In Britain, it is known as MND (Motor Neuron Disease) and in France - Maladie de Charcot.
A.L.S affects the peripheral motor nerve cells which the brain uses to supervise most voluntary mussels of the body. The nerve cells are destroyed and are unable to control muscle movement, which leads to the weakening and eventual paralysis of the muscle and the spinal cord. 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 death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed. In the course of the disease, the muscles responsible for stimulating the limbs, swallowing, speech and breathing are gradually damaged, in random order.
Five to ten percent of all ALS cases are hereditary, with more than one case per family. In most cases, the disease attacks sporadically; there is no method of predicting who will become sick.
Initial symptoms are typically minor and unspecific, a fact that usually delays diagnosis. These symptoms include:
• Weakening of the limbs' muscles.
• Muscle spasms and "jumps", especially hand and leg muscles.
• Difficulty of speech and sound.
• Difficulty in swallowing and breathing.
Every patients experience different order of symptoms and advancement in the muscle systems. However, most patients first suffer muscle weakening, which leads to tumbles, dropping of objects and fatigue.
The progression of the disease and spread of paralysis usually damages speech, swallowing, chewing and breathing.
Since the disease only harms the motor nerves, and the sensory nerves remain intact, there is no affect on sight, hearing, feeling, taste and olfactory senses. Since there is no cognitive damage, most patients remain lucid throughout the disease.
The disease attacks 1 in 10,000 people. It is estimated that 600-700 ALS patients live in Israel. The disease typically appears at ages 46-65, although there are cases of younger and older patients.
Five to ten percent of the cases concern hereditary dominance.
Most patients will live 3-5 years from the day they were diagnosed; 20 percent 5-10 years and 10 percent more than 10 years.
A.L.S is suspected when a person complains of muscle weakness that deteriorates over time, including the use of limbs and the ability to swallow and breathe. A neurological test is crucial in diagnosing the disease. The neurologist will seek proof that the motor nerves are malfunctioning.
The typical test is an EMG – which checks the functionality of motor nerves – by inserting a needle inside the weakening muscles and recording their activities.
In most cases, more tests are required to dismiss other reasons that the muscles should weaken, including blood tests, MRI and the fluid of the spinal column.
Rilozol/Rilotek is the only drug with a proven record of slowing down – by a few months – the progression of the disease. This drug has no significant side effects.
Proper nutrition and exercise (during the initial stages of the disease) were shown as effective in maintaining the patient's condition. In some cases, a gastrostom, direct feeding into the stomach, to complement or replace typical nourishment.
As the disease progresses, there is typically a need for a mechanism to support breathing. The patient can typically use non-invasive breathing instruments until advanced stages of the disease.
With A.L.S, supportive treatment is critical in maintaining the patient's quality of life. Such supportive treatment includes physiotherapy, therapy for swallowing and talking, breathing therapy and emotional therapy. Many treatments are designed to ease such symptoms as muscle cramps, shortness of breath, accumulation of saliva, pains, sleeplessness, heightened laughter or weeping and psychological difficulties.
Many accessories and appliances are available to ease upon movement and communications.