Amyotrophic lateral sclerosis (ALS) is a terminal neurological disorder characterized by progressive degeneration of nerve cells in the spinal cord and brain.
Amyotrophic lateral sclerosis is a terminal neurological disorder characterized by progressive degeneration of nerve cells in the spinal cord and brain. Often referred to as Lou Gehrig's disease (a famous baseball player who died from the disease), it is one of the most devastating of the disorders that affects the function of nerves and muscles.
ALS does not affect mental functioning or the senses (such as seeing or hearing), and it is not contagious. Currently, there is no cure for amyotrophic lateral sclerosis.
According to the ALS Association, the following statistics currently apply to ALS:
Most people who develop ALS are between the ages of 40 and 70, although the disease can occur at a younger age.
The average age for the onset of ALS is 55.
ALS occurs throughout the world with no racial, ethnic, or socioeconomic boundaries.
ALS affects as many as 30,000 Americans with 5,000 new cases diagnosed in the U.S. each year.
There are three known classifications of ALS, including the following:
Sporadic. The most common form of ALS in the U.S., involving 90 to 95 percent of all cases. These cases occur randomly, without any known cause, and there is no association with persons in the family with the disease.
Familial. Suggests that the disease is inherited and accounts for a very small number of cases in the United States, about 5 to 10 percent.
Guamanian. An extremely high incidence of ALS was observed in Guam and the Trust Territories of the Pacific in the 1950s.
Patients who suffer from ALS initially experience weakness in one of their limbs that develops over a matter of days or, more commonly, a few weeks. Then, several weeks to months later, weakness develops in another limb. Sometimes the initial problem can be one of slurred speech or difficulty swallowing.
As ALS progresses, though, more and more symptoms are noticed. The following are the most common symptoms of ALS. However, each individual may experience symptoms differently. Symptoms may include:
Twitching and cramping of muscles, especially those in the hands and feet
Loss of motor control in the hands and arms
Impairment in the use of the arms and legs
Tripping and falling
Uncontrollable periods of laughing or crying
Slurred or thick speech and difficulty in projecting the voice
As the disease progresses, symptoms may include:
The symptoms of ALS may resemble other conditions or medical problems. Always consult your doctor for a diagnosis.
In addition to a complete medical history and physical examination, diagnostic procedures for ALS may include the following:
Laboratory tests (including blood and urine studies and thyroid functioning tests)
Muscle and/or nerve biopsy. A procedure performed to remove tissue or cells from the body for examination under a microscope
Spinal tap (also called a lumbar puncture). A special needle is placed into the lower back, into the spinal canal; This is the area around the spinal cord; The pressure in the spinal canal and brain can then be measured. A small amount of cerebral spinal fluid (CSF) can be removed and sent for testing to determine if there is an infection or other problems. CSF is the fluid that bathes the brain and spinal cord.
X-ray. A diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film
Magnetic resonance imaging (MRI). A diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body
Electrodiagnostic tests, such as electromyography (EMG) and nerve conduction study (NCS). Studies that evaluate and diagnose disorders of the muscles and motor neurons. Electrodes are inserted into the muscle, or placed on the skin overlying a muscle or muscle group, and electrical activity and muscle response are recorded.
Specific treatment for ALS will be determined by your doctor based on:
Your age, overall health, and medical history
Extent of the disease
Your tolerance for specific medications, procedures, or therapies
Expectations for the course of the disease
Your opinion or preference
For most people with ALS, primary treatment may involve the management of symptoms, and may include physical, occupational, speech, respiratory, and nutritional therapies. Some medications and/or heat or whirlpool therapy may help to relieve muscle cramping. Exercise, although recommended in moderation, may help to maintain muscle strength and function.
There is no proven treatment for ALS. However, the FDA approved Rilutek, the first drug that has prolonged the survival of persons with ALS.
Managing the symptoms of ALS is a process that may be challenging for people with the condition, their caregivers, and the medical team. However, it is important to know that there are many community resources available for support and assistance.
Researchers are conducting studies to increase their understanding of genes that may cause the disease, mechanisms that can trigger motor neurons to degenerate in ALS, and approaches to stop the progress leading to cell death.
The following related clinical trials and research studies are currently seeking participants at Massachusetts General Hospital. Search for clinical trials and studies in another area of interest.
Dr. Leigh Hochberg from the Department of Neurology at Massachusetts General Hospital is using neurotechnology to harness brain signals that accompany movement. The ultimate goal of the work is to "turn thought into action," with the hope of one day assisting people with ALS, spinal cord injury and stroke to regain control over their environment.
Merit Cudkowicz, MD, director of the Mass General Neurological Clinical Research Institute, talks about clinical trials for Amyotrophic Lateral Sclerosis, also known as ALS, or Lou Gehrig's disease.
Military veterans who are diagnosed with ALS are eligible for service-connected benefits.
NEALS and ALSA partner to offer clinical trial liaison service to help people with ALS navigate the world of clinical research.
The Northeast ALS Consortium (NEALS) website is devoted to supporting clinical research of Amyotrophic Lateral Sclerosis (ALS) and other motor neuron disease (MND).
Formal statement on the forthcoming US Brainstorm trial
Mass General received a $20 million grant to establish a nationwide clinical coordinating center for testing drugs that can help people with diseases of the brain such as Alzheimer’s disease.
Biogen Idec has announced that the EMPOWER trial has failed to demonstrate efficacy in primary and secondary endpoints.
The MGH ALS Multidisciplinary Clinic and the Northeast ALS Consortium participated in the Ice Bucket Challenge on Aug. 8.
Congratulations to Dr. Merit Cudkowicz, MGH Neurology Department Chairperson and Director, MGH ALS Clinic, for receiving the MDA LOU GEHRIG HUMANITARIAN AWARD. This award is given to a researcher, physician, or clinician who supports MDA/ALS research initiatives and clinical services and continues to fight the battle against ALS. View her acceptance speech where she discusses reasons for hope in the future of ALS treatments.