Health Library: Amyotrophic Lateral Sclerosis (ALS)
Amyotrophic lateral sclerosis (ALS) affects the motor neurons connecting the brain and spinal cord. It progressively deteriorates the motor neurons reaching from the brain to the spinal cord, which eventually leads to their death. Once the neurons controlling mobility die, the brain loses the ability to control muscle movement. The progressive degeneration of ALS may leave patients completely paralyzed in its latter stages.
Amyotrophic lateral sclerosis (ALS) patients suffer from lack of muscle nourishment. “Amyotrophic” literally means “no muscle nourishment” as defined in the ancient Greek language. If muscles do not obtain adequate nutrition, then it atrophies or wastes away. “Lateral” directs attention to the spinal cord areas of the afflicted patient, usually where the concentration of nerves signaling the control of muscles are located. As a result of nerve generation within the spinal cord, scarring and hardening of the tissues in the region, often referred to as “sclerosis.”
Once motor neurons completely degenerate, they can no longer send electrical impulses to the muscle groups that cause movement. The beginning stages of ALS can produce symptoms including muscle weakness affecting the patient’s ability to move their arms, legs, speak, swallow, and breathe. All strophic muscles lose functionally once the motor neurons cease firing messages to the individual muscle groups. In fact, friends and family will begin to notice that the patient’s limbs will appear thinner as muscle tissue begins to waste away.
What is Amyotrophic Lateral Sclerosis (ALS)?
ALS affects the motor neuron system more than any other degenerative disease. Jean-Martin Charcot, a French neurologist, was the first to describe the nature of the disease in 1869; however, ALS did not garner attention until Major League Baseball hall-of-famer Lou Gehrig announced his diagnosis in 1939. Therefore, ALS has alternative names associated with these famous people, such as Charcot’s and Lou Gehrig’s disease. Some medical professionals have referred to ALS as motor neuron disease (MND).
ALS occurs when the messages or electrical impulses do not fire from the spinal cord, which causes the muscles to lose strength and wither away. This process is commonly known as atrophy. Once the signals from the brain to the spinal cord slows down, then the muscles become slow or non-responsive. This process is often referred to as spasticity. Statistically, 7 out of every 100,000 people will develop ALS between the ages of 50 and 75 years old. An estimated 10 percent of people afflicted with ALS have a family history of the disease. Patients can expect to live between 2 and 5 years after diagnosis.
- Amyotrophic Lateral Sclerosis: An overview of ALS, also known as Lou Gehrig disease, a disease affecting the motor neurons connecting the brain and spinal cord, which control voluntary muscle movement.
- Yale School of Medicine: Amyotrophic Lateral Sclerosis (ALS ): A extensive background on ALS, including its history, epidemiology, clinical features, pathophysiology, diagnosis, related disorders, genetics, treatment, drug treatment, research, and support groups.
- Sporadic ALS: A comprehensive resource detailing all aspects of sporadic ALS, including its clinical features, diagnostic criteria, differential diagnosis, epidemiology, laboratory features, pathology, prognosis, susceptibility loci, and treatment methods.
- Health Conditions: Amyotrophic Lateral Sclerosis (ALS): An estimated 30,000 patients within the United States have been diagnosed with Amyotrophic Lateral Sclerosis (ALS), a disease named after the baseball hall of famer, Lou Gehrig.
- Studies and Participation: Lou Gehrig’s Disease: An educational resource detailing the studies and participation involved in ALS, including how genetics are involved in the formation of the disease, ALS research reviews, ALS publications, and miscellaneous ALS information.
- Amyotrophic Lateral Sclerosis for Families: A family-oriented introduction to ALS, which includes information for families with relatives afflicted with Lou Gehrig’s disease. The basic information contains instructions on who to contact, where to go for support, where to learn more about the disease, and what Internet websites offer helpful advice.
- ALS and Neuromuscular Center: Amyotrophic Lateral Sclerosis, a neuromuscular disease, is characterized by muscle weakness and sensory disturbances.
- What is ALS?: Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, Motor Neuron Disease (MND), and Charcot’s disease, affects the motor neurons within the brain and spinal cord.
- Amyotrophic Lateral Sclerosis (Upper and Lower Neurons): Amyotrophic lateral sclerosis (ALS) primarily affects the upper (brain) and lower (spinal cord) neurons that control motor function within the body.
- Amyotrophic Lateral Sclerosis (ALS) Background: Emedicine provides background information on ALS, a neurodegenerative and fatal disease.
Symptoms, Causes, and Diagnosis
Researchers still have not discovered the cause of ALS; however, between five and ten percent of all cases stem from genetics. Some research has connected ALS with Alzheimer’s, Parkinson’s, and other neurodegenerative diseases. Lou Gehrig’s disease typically affects the motor neurons connected throughout multiple regions in the body. Progressive degeneration occurs in both the lower and upper more neurons. Lower motor neurons are located near the spinal cord, whereas upper motor neurons are connected to the brain. The loss of lower motor neurons leads to the loss of mobility through muscle atrophy. The loss of upper motor neurons produces spasticity, abnormally active and pathological reflexes. Additionally, the loss of upper neurons may lead to cognitive impairment as a result of damage to the prefrontal neurons. This can lead to maladaptive social behaviors, or frontotemporal dementia.
Classic ALS involves the neurodegeneration of both upper and lower motor neurons. Sporadic ALS usually starts as a weakness in one region before spreading gradually throughout the rest of the body. If only lower motor neurons are damaged, the disease is referred to as progressive muscular atrophy (PMA). If only the upper motor neurons are involved, then the patient diagnosis is primary lateral sclerosis (PLS). The clinical diagnosis of ALS can be administered with electrodiagnostic testing devices. Morbidity usually occurs from aspiration pneumonia and other medical complications related to mobility.
- Disease Fact Sheets: Amyotrophic Lateral Sclerosis: A fact sheet addressing the symptoms, causes, diagnosis, and treatment of ALS. It also includes the genetic background, progression, drug treatments for the degenerative disease.
- Rehabilitation Medicine: Amyotrophic Lateral Sclerosis (ALS): The University of Washington’s rehabilitation center discusses the symptoms, causes, risk factors, diagnosis, complications, and treatments of ALS.
- Amyotrophic Lateral Sclerosis (ALS): A definitive essay focused on the direct causes of Lou Gehrig’s disease, including the underlying causes, symptoms, and means of diagnosing the illness.
- The Ohio State University Medical Center: Amyotrophic Lateral Sclerosis: Ohio State’s Medical Center answers frequent asked questions regarding ALS, including the various types, symptoms, diagnosis, and treatment for the neurodegenerative disease.
- ALS Signs, Symptoms and Diagnosis: A small and concise summary explaining what signs to look for in an ALS patient. A proper diagnosis can be assessed according the patient’s symptoms as confirmed by the medical doctor.
- Amyotrophic Lateral Sclerosis (ALS) (PDF): An extensive fact sheet answering commonly asked questions of various patients and their families in regards to ALS, including what signs to identify the underlying problem, the diagnosis procedure, various treatment methods, and important lifestyle changes needed to better cope with the disease.
- Amyotrophic Lateral Sclerosis (ALS) FAQ: The American Speech-Language-Hearing Association answers frequently asked questions about ALS, including what and how it came into existence, identifiable signs and symptoms, diagnostic procedures and tools used on suspected ALS patients, available treatments, and finding a good support group or organization to help patients cope with the illness.
- Lou Gehrig’s Disease: The Mayo Clinic offers a brief overview, diagnosis information, treatment methods, appointments, clinical trials, and research in regards to Lou Gehrig’s disease.
- ALS Information: The Charlie Wedemeyer Family Outreach Center answers 16 questions asked by students and patients in regards to ALS, including the definition of ALS, life expectancy rate, symptoms to identify, the recurring rate of the disease, liability factors, and whether or not its contagious.
- John Hopkins Medicine: Amyotrophic Lateral Sclerosis: The John Hopkins Medical Center provides an overview of the neurodegenerative disease, including ALS statistics, the underlying causes of ALS, the individual symptoms of Lou Gehrig’s disease, ALS diagnostic tests, and learning to identify the different types of ALS.
Treatment and Research
Clinical research studies addressing Lou Gehrig’s disease involves people, but rarely includes experimental drugs or devices. These studies help doctors, researchers, and scientists learn more about the disease, and potentially discover more sophisticated treatment options. As of today, Lou Gehrig’s disease remains incurable; however, conducted studies have the potential to lead to future developments. Research studies have targeted genetic causes of the disease in families with a track record of ALS. This focus primarily investigates whether some ALS patients have more of a genetic predisposition to the disease than other patients with a non-inherited track record. Additionally, research facilities have investigated new techniques for diagnosing the neurodegenerative disease.
Most clinicians will prescribe medicine called riluzole to help prolong the lifespan of the patient; however, it will not reverse or halt the progress of the disease. The primary goal of treatment is to control symptoms with prescription drugs, such as baclofen or diazepam, which control both spasticity and the ability to swallow one’s food or saliva. Physical therapy may include the use of braces, a wheelchair, or other orthopedic apparatus to help with mobility. ALS patients may experience problems with choking, which may result in having a feeding tube placed in their stomach. A nutritionist may also prescribe a certain diet to help prevent weight loss. Some patients will need breathing apparatuses in the latter stages of the illness.
- ALS Therapy Alliance: Over a decade, the ALS Therapy Alliance research program has sought a cure for Lou Gehrig’s disease. The organization has raised over $26 million to support this cause, because of its partnership with CVS/pharmacy.
- Discovery Offers New Therapy Approach for Lou Gehrig’s Disease: Researchers from Uruguay and Oregon State University discovered an important neural cell closely linked with the progression of ALS.
- Current Projects in ALS and Motor Neuron Disorder Research: UC San Diego School of Medicine has been developing a cell replacement therapy for ALS, identifying an FDA-approved drug for slowing ALS progression, creating a paradigm shift in conventional diagnosis, developing gene slicing therapy for ALS patients, and developing viral gene therapy for ALS.
- Gene Therapy Postpones Lou Gehrig’s Disease Symptoms: A case study reveals that gene therapy postpones the progression of Lou Gehrig’s disease symptoms. In fact, the gene therapy nearly doubles the life span of a mouse in an animal model.
- Exercise Plus Gene Therapy Shows Promise for Treating Lou Gehrig’s Disease: A combination of both gene therapy and regular exercise shows promise in alleviating the severity of Lou Gehrig’s disease. In fact, the relation between gene therapy and regular exercise may prove promising in future efforts to slow down the progression of ALS.
- Research Collaboration Targets Stem Cell Therapy for Lou Gehrig’s Disease: A team of researchers from the University of Utah collaborated in an effort to use stem cell therapy for the treatment of Lou Gehrig’s disease.
- Breakthrough Could Lead to Effective Treatments for Lou Gehrig’s Disease: Northwestern University’s researchers have discovered a common cause behind ALS, which could potentially lead to effective treatment against Alzheimer’s, dementia, and Parkinson’s disease.
- Phytopharm’s Treatment for Lou Gehrig’s Disease Gains Orphan Drug Status in EU: A new prescription drug treatment with Phytopharm has gained orphan drug status in the European Union.
- Amyotrophic Lateral Sclerosis (ALS) Treatment: The University of California of San Francisco lists several treatment options for patients suffering with ALS, including prescription drugs, such as baclofen and diazepam to help control spasticity, gabapentin for pain relief, and trihexyphenidyl or amitriptyline to help patients swallow their own saliva.
- Forbes Norris MDA/ALS Research & Treatment Center at CPMC: An award-winning research and treatment center for motor neuron diseases.