ALS, also known as amyotrophic lateral sclerosis or Lou Gehrig’s disease, is a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord which causes the loss of muscle control. Eventually, people with ALS lose the ability to initiate and control muscle movement, which often leads to total paralysis and death within two to five years of diagnosis. Although there is currently no cure and no life-prolonging treatments for the disease, a recently FDA-approved medication has been demonstrated to slow the decline of various physical activities.
Although the cause of ALS is unknown, researchers continue to study the possible causes. It is believed that both genetics and environment play a role in the development of ALS. There are two types of ALS – familial and sporadic.
According to experts, familial ALS (FALS) is extremely rare and only accounts for 5 to 10 percent of all ALS cases. FALS is inherited and is most often autosomal dominant. In FALS cases, a parent with the genetic mutation responsible for the disease has a 50 percent chance of passing the mutation to each of their children. The genetic mutation responsible for FALS is equally likely to be inherited by men and women, can exist at various ages, and progress differently among family members.
Sporadic ALS is the most common form of ALS and accounts for 90 to 95 percent of all cases. Sporadic ALS cases occur randomly, lack a known cause, and have no family history of ALS. Scientists have proposed a chemical imbalance, an overactive immune response, and protein mishandling as other possible causes of non-inherited forms of ALS.
ALS kills the motor neurons that run from the brain to the spinal cord and to the muscles throughout the body. The brain is not able to initiate and control muscle movement. ALS signs and symptoms vary among individuals, although advanced muscle weakness and paralysis are commonly experienced. Additionally, symptoms are not experienced in the same sequence or patterns of progression.
ALS characteristically involves a gradual onset. Muscles will begin to weaken and eventually paralyze. Early symptoms of ALS include:
The onset of ALS involves muscle weakness or stiffness. Ultimate progression of paralysis of the muscles of limbs and trunk, as well as those that control vital functions such as speech, swallowing, and breathing typically follows. The progression of ALS can make everyday tasks very difficult – such as grasping objects or even walking. The stages of ALS vary for each person and progression is not always linear. The average survival time for a person diagnosed with ALS is two to five years. About 10 percent will survive 10 years, and 5 percent will live for 20 years or longer.
Those diagnosed with ALS could encounter periods of time where there is very little to no loss of function, or there is a significant improvement and recovery of lost function. These periods are known as “arrests” and “reversals” and can last from weeks to months at a time. It is important to note that these periods are very rare and less than 1 percent of people with ALS will have significant improvement in function lasting 12 months or longer.
There is not yet a cure for ALS nor an effective treatment to halt or reverse the progression of the disease. However, there are a variety of treatments to help manage symptoms, prevent complications, and assist people living with ALS. There is no standard treatment for ALS and treatment is based on the symptoms of each person diagnosed with ALS.
Currently, there are four drugs approved by the United States FDA to treat ALS – Riluzole, Nuedexta, Radicava, and Tiglutik. Additionally, studies have shown that participation in multidisciplinary ALS clinics may improve quality of life and prolong survival.
An early, accurate diagnosis and receiving the proper treatment in a timely fashion are essential in the fight against ALS. Roseman Medical Group (RMG) has an electromyography (EMG) laboratory that aids in the diagnosis of ALS and a comprehensive ALS clinic that supports individuals diagnosed with ALS.
The Roseman Medical Group ALS Clinic, southern Nevada’s second clinic, is dedicated to treating those diagnosed with ALS. The ALS Clinic provides evidence-based, multidisciplinary ALS care and services in a supportive atmosphere with an emphasis on hope and quality of life.
Through the multidisciplinary ALS Clinic, RMG neurologist David Ginsburg, M.D. and a team of highly qualified healthcare professionals work together to address the needs of individuals living with ALS. The Clinic provides care to six patients during each monthly half-day clinic in which each patient spends about three hours receiving care from a variety of specialists based on their individual needs. During a given clinic visit a patient can expect to receive access to a speech therapist, occupational and physical therapist, respiratory therapist, dietitians, nursing/social services, and others to assist them in living with the disease. The Clinic helps in eliminating the time and cost associated with having to visit multiple clinics for numerous appointments with various specialists, streamlining care and allowing for treatment of the whole patient.
Our neurologists provide empathic, patient-centered, evidence-based care. If you have a friend or family member who suffers from ALS, let RMG’s expert neurologists help! Contact us today!
This article was originally posted on Roseman University of Health Sciences’ blog, as well as published in the May/June edition of Roseman University’s print edition