Amyotrophic lateral sclerosis

1. Windsor University School of Medicine developed multiple teaching slides in both basic medical sciences, clinical medical sciences and USMLE course work. These teaching material will be available for its students to access. Sample teaching slide

2. Amyotrophic lateral sclerosis Amyotrophic lateral sclerosis (ALS) is a serious neurological disease that attacks the nerve cells that control voluntary muscles. It may begin with muscle twitching or weakness in an arm or leg, or with slurring of speech. Eventually, amyotrophic lateral sclerosis affects your ability to control the muscles needed to move, speak, eat and breathe.

3. Signs and symptoms • Signs of amyotrophic lateral sclerosis are typically subtle at first. The disease commonly begins in one part of the body and progresses to other areas. Gradual death of nerve cells saps muscles of their control. Early signs and symptoms of ALS include: • Difficulty lifting the front part of your foot (footdrop) • Weakness in your leg, feet or ankles • Hand weakness or clumsiness • Slurring of speech or trouble swallowing • Muscle cramps and twitching in your arms, shoulders and tongue • Fatigue in combination with the above signs and symptoms

4. Causes • Inherited (familial) ALS. Up to one in 10 cases of ALS appear to be inherited. About 20 percent of these cases result from a specific gene mutation in an enzyme known as superoxide dismutase 1 (SOD1). Scientists and researchers are still trying to identify other ALS gene mutations that may be inherited. • Sporadic ALS. More than 90 percent of cases of ALS appear to occur randomly, with no identifiable cause and no obvious risk factors.

5. Risk factors • Although most cases of amyotrophic lateral sclerosis have no apparent cause, a small percentage of people who have ALS inherit it. Inherited ALS may strike at a younger age and will affect consecutive generations. If you're the first person in your family to have amyotrophic lateral sclerosis, you likely don't have an inherited form, and your children aren't at a greater risk. • Noninherited ALS often develops between ages 40 and 60, although the disease affects people of all ages. ALS affects more men than women.

6. Screening and diagnosis • Electromyogram (EMG). Electromyography measures the tiny electrical discharges produced in muscles. A thin-needle electrode is inserted into the muscles that your doctor wants to study. An instrument records the electrical activity in your muscle as you rest and contract the muscle. • Nerve conduction study (NCS). As a part of an electromyography study, two electrodes are taped to your skin above a nerve or muscle to be studied. A small shock is passed through the nerve to measure the size and speed of nerve signals. • Imaging. Your doctor may suggest magnetic resonance imaging (MRI) or a computerized tomography (CT) scan to produce images of your brain and spinal cord. These tests can help your doctor determine if something other than ALS may be causing your signs and symptoms. • Spinal tap (lumbar puncture). This test analyzes the fluid surrounding your brain and spinal cord (cerebrospinal fluid). You typically lie on your side with your knees drawn up to your chest. A local anesthetic is injected in an area over your lower spine to reduce any discomfort from the procedure. Then a needle is inserted into your spinal canal, and fluid is collected. Tests on this fluid indicate the number and types of white blood cells, the level of sugar, and the levels and types of proteins. Additional tests on this fluid can indicate evidence of bleeding, infection and abnormal cells.

7. Blood and urine tests. Analyzing samples of your blood and urine in the laboratory may help your doctor eliminate other possible causes of your signs and symptoms. • Muscle biopsy. If your doctor believes you may have a muscle disease rather than ALS, you may undergo a muscle biopsy. In this procedure, a small portion of tissue is removed while you're under local anesthesia and is sent to a lab for analysis.

8. Treatment • Medications. The drug riluzole (Rilutek) is the first and only medication approved by the Food and Drug Administration for slowing ALS. The drug appears to slow the disease's progression and prolong life by a few months in some people, perhaps by reducing glutamate levels. Your doctor may also prescribe medications to provide relief from muscle cramps and constipation and to reduce fatigue, excessive salivation and excessive phlegm. Some people with amyotrophic lateral sclerosis also benefit from medications for pain or depression. • Physical and occupational therapy. A physical therapist can recommend low-impact exercises to maintain your muscle strength and range of motion as long as possible, helping you preserve a sense of independence. An occupational therapist can help you become accustomed to a brace, walker or wheelchair and may be able to suggest devices such as ramps that make it easier for you to get around. • Speech therapy. Because ALS affects the muscles you use to speak, communicating clearly becomes an issue as the disease progresses. A speech therapist can help teach techniques to make your speech more clearly understood. Later in the disease, a speech therapist can recommend devices such as speech synthesizers and computers that may help you communicate. • Nutritional support. A dietitian can advise you on foods that are nutritious but easy to swallow. As the disease progresses and you have greater difficulty swallowing, you may consider a feeding tube inserted into your stomach to receive nutrition and avoid choking.