Exercise as it relates to Disease/Exercise and its effects on Amyotrophic Lateral Sclerosis (ALS)

What is Amyotrophic Lateral Sclerosis?
Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig's disease in the United States, is the most common adult motor neuron disease. The disease is rapidly progressive and invariable fatal as it attacks the nerve cells (neurons) responsible for controlling voluntary muscles (various muscle actions we have control over, such as those in our arms, legs and face). Since ALS is characterized by the gradual degeneration and death of motor neurons, it is categorized into a group of disorders known as the motor neuron diseases.

Prevalence
The average incidence of ALS is 2 cases per 100,000/year. The actual prevalence being 6 per 100,000/year, which is 2 to 3 times greater than the incidence.

Causes of ALS
Approximately 85-90% of cases are sporadic, meaning they have no genetic component, the remaining 5-10% of cases are inherited (familial). So far 11 genes have been identified as associated with the disease. The most studied gene encodes for copper/zinc superoxide dismutase enzyme (SOD1). Both forms of ALS are clinically and pathologically similar. The typical age for both forms is between 50 and 60 years. Causes for most cases of ALS are unknown and the clinical course is highly variable, suggesting that multiple factors underlie the disease mechanism.

Mutated SOD1
The most widely researched mutation leading to ALS is the cystolie antioxidant enzyme Cu/Zn-SOD (SOD1). 20-30% of familial ALS (FALS) cases are caused by the dominant inheritance of mutations in the SOD1 gene. These mutations occur outside the active site of the SOD1 enzyme, modify the stability of the protein backbone, and lessen the enzyme's affinity of Zinc Though it is still not clear as to how mutations in the SOD1 gene lead to motor neuron degeneration, increasing evidence suggests that mutant SOD1 protein can become toxic.

Effects of ALS
The disease's primary characteristic is the selective dysfunction and death of the neurons in the motor pathways. The shutdown of these neurons lead to: Failure of the respiratory muscles is generally the fatal event, occurring within one to five years of the onset of the disease. If symptoms occur in the arms or legs first, it is referred to as "limb onset" ALS. Other patients first notice speech problems, this is termed "bulbar onset" ALS.
 * Fasciculations, cramps, tight and stiff muscles (spasticity)
 * Hyperreflexia (exaggerated reflexes) including an overactive gag reflex - usually occurring in the upper motor neurons
 * Generalized weakness
 * Muscle atrophy
 * Paralysis - usually occurring in the lower motor neurons
 * Slurred and nasal speech, or difficulty chewing or swallowing

Recommendations for Effective Exercise Rehabilitation
Before undertaking any exercise, patient must be prescreened. Any medications, strength, range of motion, level of fitness must be taken into consideration while developing a program for ALS patient. Ensure program and designated activities is cleared by a physician or physiotherapist, and that all activities are supervised.

Improving Compliance

 * Involvement, support and interaction from patient's family, community and healthcare professionals
 * Providing a positive outlook and interest in prescribes exercises will increase levels of motivation
 * Altering activities to reduce exercise-related pain
 * Prescribing activities that suit the patient's interests
 * Creating a programs that suits the patient's daily schedule.