Lou Gehrig disease; ALS; Upper and lower motor neuron disease; Motor neuron disease DefinitionAmyotrophic lateral sclerosis, or ALS, is a disease of the nerve cells in the brain, brain stem and spinal cord that control voluntary muscle movement. ALS is also known as Lou Gehrig disease. CausesOne in 10 cases of ALS is due to a genetic variant. The cause is unknown in most other cases. In ALS, motor nerve cells (neurons) waste away or die, and can no longer send messages to muscles. This eventually leads to muscle weakening, twitching, and an inability to move the arms, legs, and body. The condition continues to get worse. When the muscles in the chest area stop working, it becomes hard or impossible to breathe. ALS affects approximately 7 out of every 100,000 people worldwide. Having a family member who has a hereditary form of the disease is a risk factor for ALS. Other risks include military service. The reasons for this are unclear, but it may have to do with environmental exposure to toxins. SymptomsSymptoms usually do not develop until after age 50, but they can start in younger people. People with ALS have a loss of muscle strength and coordination that eventually gets worse and makes it impossible for them to do routine tasks such as going up steps, getting out of a chair, or swallowing. Weakness can first affect the arms or legs, or the ability to breathe or swallow. As the disease gets worse, more muscle groups develop problems. ALS does not affect the senses (sight, smell, taste, hearing, touch). Most people are able to think normally, although a small number develop dementia, causing problems with memory. Muscle weakness starts in one body part, such as the arm or hand, and slowly gets worse until it leads to the following:
Other findings include:
Exams and TestsYour health care provider will examine you and ask about your medical history and symptoms. The physical exam may show:
Tests that may be done include:
TreatmentThere is no known cure for ALS. Two medicines are available that help slow the progression of symptoms and may help people live slightly longer:
Treatments to control other symptoms include:
Trials of gene therapy are now underway for some specific hereditary causes. Physical therapy, rehabilitation, use of braces or a wheelchair, or other measures may be needed to help with muscle function and general health. Ongoing oversight by a physical medicine provider is helpful, given continuously changing symptoms. People with ALS tend to lose weight. The illness itself increases the need for food and calories. At the same time, problems with choking and swallowing make it hard to eat enough. To help with feeding, a tube may be placed into the stomach. A dietitian who specializes in ALS can give advice on healthy eating. Breathing devices include machines that are considered non-invasive (such as CPAP or BiPAP). Others require tube in the trachea (invasive ventilation). Medicine for depression may be needed if a person with ALS is diagnosed with depression. They also should discuss their wishes regarding artificial ventilation with their families and providers. Support GroupsEmotional support is vital in coping with the disorder, because mental functioning is not affected. Groups such as the ALS Association may be available to help people who are coping with the disorder. Support for people who are caring for someone with ALS is also available, and may be very helpful. Outlook (Prognosis)Over time, people with ALS lose the ability to function and care for themselves. Death often occurs within 3 to 5 years of diagnosis. About 1 in 4 people survive for more than 5 years after diagnosis. Some people live much longer, but they typically need help breathing from a ventilator or other device. Possible ComplicationsComplications of ALS include:
When to Contact a Medical ProfessionalContact your provider if:
Increased difficulty swallowing, difficulty breathing, and episodes of apnea are symptoms that require immediate attention. PreventionYou may want to see a genetic counselor if you have a family history of ALS. ReferencesFearon C, Murray B, Mitsumoto H. Disorders of upper and lower motor neurons. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds. Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier; 2022:chap 97. Shaw PJ, Cudkowicz ME. Amyotrophic lateral sclerosis and other motor neuron diseases. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 387. | ||
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Review Date: 6/13/2024 Reviewed By: Joseph V. Campellone, MD, Department of Neurology, Cooper Medical School at Rowan University, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. View References The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. No warranty of any kind, either expressed or implied, is made as to the accuracy, reliability, timeliness, or correctness of any translations made by a third-party service of the information provided herein into any other language. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited. | ||
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Central nervous sy...
