Rare and incurable, Amyotrophic Lateral Sclerosis (ALS) is also the most severe of degenerative neurological diseases. In the U.S., it is known as Lou Gehrig’s disease, named after the American baseball player who contracted the disease in the 1930’s. In Great Britain the disease is called Motor Neuron Disease (MND), and in France it is known as Maladie de Charcot.
ALS attacks the peripheral motor nerve cells used by the brain to control most of the voluntary muscles in the body. Gradually, the destruction of these nerve cells leads to impaired muscle movement, and eventually to the weakening and final paralysis of the muscle and spinal cord. With the progressive degeneration and death of motor neurons — leading from the brain to the spinal cord and from the spinal cord to the muscles throughout the body — the brain loses its ability to initiate and control muscle movement. As ALS takes its course, limb movement, swallowing, speech and breathing are gradually affected in random order and patients in the later stages of the disease may become totally paralyzed.
ALS affects approximately 1 in 10,000 individuals, Five to ten percent of all ALS cases are the result of genetic defects, and sometimes more than one member of a family is stricken. In most cases, however, the disease attacks randomly, and unpredictably. There is no way of knowing who may become a victim.
It is estimated that there are 600-700 ALS patients in Israel today. Most are between 46-65 years old but some are younger or older. Death often occurs within three to five years of diagnosis. 20% of the patients survive five to ten years and 10% live longer.
Symptoms and Diagnosis
The initial symptoms of ALS, typically minor and unspecific, make diagnosis difficult. These symptoms include:
• Weakening of the limb muscles.
• Muscle spasms and twitching, especially of hand and leg muscles.
• Difficulties in speaking
• Difficulties in swallowing and breathing.
Patients experience the first symptoms in random order, but in most cases the first sign is weakness in the limbs, which can cause the patient to trip and fall, drop objects and feel fatigued. Though speech, swallowing, chewing and breathing become difficult as the disease spreads, the sensory nerves stay intact, and patients retain their sense of sight, hearing, feeling, taste and smell. In addition, as there no cognitive damage, most patients remain lucid throughout the disease.
ALS is suspected when a patient complains of increasing muscle weakness along with difficulties in swallowing and breathing. An EMG, which checks the functionality of motor nerves, is crucial in diagnosing the disease. Additional tests are conducted to rule out other sicknesses and further confirm diagnosis.
Riluzole/Rilutek, which usually has no significant side effects, is the only drug that has been found to be successful in altering the course of ALS and prolonging the life of patients, even if only by a few months. Likewise, studies have shown that proper nutrition and exercise during the initial stages of the disease are effective in reducing further deterioration in the patient’s condition. A gastrostomy enables direct feeding, and complements or replaces regular nourishment. As the disease progresses, non-invasive breathing devices facilitate breathing and are usually effective up until the advanced stages of the disease, when permanent ventilatory apparatus are introduced. Many accessories and appliances are available, which can help facilitate movement and communications.
Supportive treatment is critical in maintaining the quality of life of ALS victims. Physiotherapy, therapy for swallowing and talking, breathing therapy and emotional therapy are some of the treatments available. These can ease symptoms such as muscle cramps, shortness of breath, accumulation of saliva, pain, sleeplessness, uncontrollable laughter or crying and psychological distress.