Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig’s disease, is a progressive neuromuscular disease.
ALS is characterized by a progressive degeneration of motor nerve cells in the brain (upper motor neurons) and spinal cord (lower motor neurons). When the motor neurons can no longer send impulses to the muscles, the muscles begin to waste away (atrophy), causing increased muscle weakness.
ALS does not impair a person’s intellectual reasoning, vision, hearing or sense of taste, smell and touch. In most cases, ALS does not affect a person’s sexual, bowel or bladder functions.
ALS is often referred to as a syndrome, because the disease itself becomes apparent in various patterns. ALS occurs rarely and without regularity.
What are the symptoms of ALS?
The following symptoms become more noticeable as ALS progresses:
- Twitching and cramping of muscles, especially those in the hands and feet.
- Impaired use of arms and legs.
- Weakness and fatigue.
- Weight loss.
- “Thick” speech and difficulty projecting voice.
These symptoms characterize ALS as the disease becomes more severe:
- Shortness of breath.
- Difficulty in breathing and swallowing.
Please note that severe pain is not a typical symptom of ALS.
Who is affected by ALS?
Throughout the world, an average of 1 to 2 per 100,000 people are diagnosed with ALS every year. Men are affected more frequently than women. Most people are diagnosed with ALS in their mid-50s, but ALS can also affect young adults in their mid-teens. However, onset of the disease before 30 years of age is rare.
What are the classifications of ALS?
Because no specific qualities can be predicted of all people diagnosed with ALS, the cause is unknown. The following classification is used by physicians:
A progressive neurological disease characterized by a deterioration of upper and lower motor nerve cells (neurons). This type of ALS affects over two-thirds of all people with ALS.
Primary Lateral Sclerosis
A progressive neurological disease in which the upper motor nerve cells (neurons) deteriorate. If the lower motor neurons are not affected within two years, the disease usually remains a pure upper motor neuron disease. This is the rarest of all forms of ALS.
Progressive Bulbar Palsy (PBP)
A condition that starts with difficulties speaking, chewing and swallowing due to lower motor nerve cell (neuron) deterioration. This disorder affects about 25% of all people with ALS.
Progressive Muscular Atrophy (PMA)
A progressive neurological disease in which the lower motor neurons deteriorate. If the upper motor neurons are not affected within two years, the disease usually remains a pure lower motor neuron disease.
A progressive neurological disease that affects more than one member of the same family. This type of ALS accounts for a very small number of people with ALS in the United States (5-10%).
How is ALS diagnosed?
A complete physical exam and an evaluation of the person’s medical history are required to properly diagnose ALS. Several laboratory and radiographic tests are also conducted to confirm the diagnosis, including:
- Electromyogram (EMG) [a test that measures the electrical activity in your nerves and muscles]
- Blood test
- Spinal fluid test
- Magnetic Resonance Imaging (MRI) [a test that produces images of the body without the use of x-rays]
How is ALS treated?
There is currently no cure for ALS, yet there are several treatment options that can make people diagnosed with ALS more comfortable.
Treatment options include:
- Medications to relieve painful muscle cramps, excessive salivation and other symptoms.
- Physical therapy to maintain mobility and ease the discomfort of muscle stiffness, cramps and fluid retention.
- Nutritional counseling to promote good nutrition and recommend other dietary options when swallowing becomes difficult.
- Speech therapy and communication training to maintain as many verbal communication skills as possible. Communication training also includes non-verbal techniques.
- Assistive devices such as splints, corrective braces, grab bars, reach devices, etc. to help with daily activities such as dressing, eating, toileting and bathing.
- Special equipment such as wheelchairs, electric beds or mattresses and boards to maximize functional independence.
What is the Care Path For People With Presumed ALS?
- Neurological evaluation is conducted
- Reasons for other tests are discussed
- Laboratory studies are conducted
- Additional testing is performed, such as an electromyogram (EMG), neuro-imaging and muscle testing
Neurological evaluation is conducted
Follow-Up Visit in 1 to 3 weeks
- Test results are discussed
- ALS diagnosis is introduced
- Services offered at The Cleveland Clinic’s Center for ALS and Related Disorders are described
- Holistic care philosophy is explained
- ALS informational booklet is provided
- Research and clinical trial opportunities are discussed
- Specific research options are explained
Specific research options are explained
- ALS patient attends the ALS Center every 3 months
- Non-IND trials are discussed as an option
Follow-Up Care For All ALS Patients
- ALS patient attends the ALS Center every 3-6 months or as needed
- Consultation with the Pulmonary Care Team is scheduled if necessary
- Consultation with a gastroenterologist is scheduled to discuss the percutaneous endoscopic gastrostomy procedure
- Patients’ legal options – including durable power of attorney and living wills – are explained
- Home care referrals are discussed
- Hospice referrals are discussed