What is ALS?
Every 90 minutes, someone in the US is diagnosed with ALS. And every 90 minutes, someone in the US dies of ALS.
ALS (Amyotrophic Lateral Sclerosis) is a rapidly progressive, terminal disease that causes muscle weakness, difficulty speaking and swallowing and, generally, complete paralysis.
There is no known cure for ALS and once diagnosed, patients typically live three to five years.
No two cases of ALS are alike. In addition, no two ALS patients are exactly alike. The symptoms and progression of the disease can vary greatly. And because decisions about treatment often affect the entire family, they must be made with their unique needs in mind.
The best course of action is to work with a multidisciplinary team of specialists trained in the treatment and management of ALS who can offer individualized care and support. That’s the approach of the clinical team at the Lois Insolia ALS Clinic at the Les Turner ALS Research and Patient Center at Northwestern Medicine, or one of the members of our Home and Community Services Team. If you or someone you know has been diagnosed with ALS, please contact Director of Patient Services, Judy Richman or call 847 679 3311.
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Questions & Answers
In ALS, motor neurons gradually cease functioning and die. As this happens, the muscle tissues waste away because no movement is being stimulated. This results in gradually worsening muscle weakness, atrophy and often spasticity. Only the motor neurons are affected. Other nerve cells, such as sensory neurons that bring information from sense organs to the brain, remain healthy.
- Weakness or poor coordination in one limb
- Changes in speaking or swallowing
- Unusual muscle twitches, spasms or cramps
- Unusual weight loss or loss of muscle bulk
Typically, ALS affects motor neurons in both the brainstem and spinal cord. Symptoms related to the brainstem neurons (sometimes referred to as “bulbar symptoms”) can include spasticity or stiffness in the lower limbs, face or jaw. Feelings of heaviness, fatigue, stiffness and lack of coordination are common. Reflexes may be very brisk or exaggerated.
Unprovoked outbursts of laughter or crying can occur, a condition often referred to as “pseudobulbar affect” or “emotional lability.” Symptoms related to the spinal cord neurons (sometimes referred to as “somatic symptoms”) can include weakness, muscle wasting or muscle twitching.
It is important to remember that ALS strikes people in different ways. The symptoms and progression of the disease will be different from person to person.
As the disease progresses, the patient usually experiences a decline in speech, swallowing and limb strength and function. Generally, ALS is not a physically painful condition, though discomfort can result from immobility and muscle shortening. The ALS patient usually remains alert and retains normal sensation, vision, bowel and bladder function. While most patients do not have loss of intellectual function, some may have subtle changes in mood, behavior or personality. In a small minority of patients, more significant changes in behavior and judgment suggest a form of dementia.
In 2011, researchers, funded in part by the Les Turner ALS Foundation, identified a common cause of all forms of ALS: a broken-down protein “recycling system” in the neurons of the spinal cord and brain. Additional research is needed to determine how best to treat this faulty pathway.
The quality of life of patients with ALS can often be improved by various treatments and interventions. Care provided by experts in multidisciplinary centers has been shown to prolong survival (Miller, et. al., 2009). Proper positioning, exercise, physical therapy and medications can help patients manage their symptoms. A feeding tube may be suggested if there is inadequate nutrition, extended meal times, rapid weight loss, high risk of aspiration (inhaling food or liquids into the lungs) or recurrent pneumonia. A wide range of devices and techniques can address problems with communication. Ultimately, ALS may result in sleep interruptions and changes in breathing, requiring consideration of airway clearance therapies. This may range from medications to non-invasive (mask) ventilation to a tracheostomy with mechanical ventilation. Managing symptoms is often a full-time responsibility for the patient and their caregivers. Connecting with a clinic such as the Lois Insolia ALS Clinic at the Les Turner ALS Research and Patient Center helps patients and their families to coordinate care in the best way.
