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What is Amyotrophic Lateral Sclerosis (ALS)? Edit

ALS, or Amyotrophic Lateral Sclerosis, is a progressive neurodegeneractive disease that affects nerve cells in the brain and the spinal cord. A-myo-trophic comes from the Greek language. "A" means no. "Myo" refers to muscle, and "Trophic" means nourishment. Amyotrophic together means "No muscle nourishment". When a muscle has no nourishment, it wastes away. This is a terminal disease. ALS does not have a cure or an effective treatment.

1. The disease I chose is ALS. It is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells responsible for controlling voluntary muscles (muscle action we are able to control, such as those in the arms, legs, and face).

ALS, or amyotrophic lateral sclerosis, is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Amyotrophic comes from the Greek language and means “No muscle nourishment.” When a muscle has no nourishment, it wastes away. “Lateral” identifies the areas in a person’s spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates it leads to scarring or hardening (“sclerosis”) in the region.

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Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body.  The progressive degeneration of the motor neurons in ALS eventually leads to their demise. When the motor neurons die, the ability of the brain to initiate and control muscle movements is lost. With involuntary muscle action progressively affected, people may lose the ability to speak, eat, move and breathe. The motor nerves that are affected when you have ALS are the motor neurons that provide voluntary movements and muscle control.

How does ALS Affect the Nervous System? Edit

2. The disease belongs to a group of disorders known as motor neuron diseases. Motor neurons are nerve cells located in the brain, brain stem, and spinal cord that serve as controlling units and vital communication links between the nervous system and the voluntary muscles of the body.

ALS severely affects the Nervous System. The Nervous System sends signals to the entire body and controls the functions and movements of the body. ALS affects motor neurons which are found in the brain and therefore affect the signals the brain sends to the body. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. ALS causes progressive degeneration of motor neurons and this leads to their demise. When the motor neurons die, the ability of the brain to conduct and control muscle movements is gone. When the muscle action in the body is affected, this causes people to possibly lose the ability to speak, eat, move and breathe. ALS affects the brain’s control on the body’s muscle movements and causes motor neurons to die, which leads to the person facing much more difficulty to perform simple everyday tasks. 

Signs and Symptoms Edit

3. Early Symptoms include fasciculation’s, cramps, tight and stiff muscles (spasticity), muscle weakness affecting an arm or a leg, slurred and nasal speech, or difficulty chewing or swallowing. Long term affects include muscle weakness and atrophy spread to other parts of the body as the disease progresses. Individuals may develop problems with moving, swallowing, and speaking or forming words. Symptoms of upper motor neuron involvement include spasticity and exaggerated reflexes including an overactive gag reflex.

Early Symptoms Can Be:

  • Pain
  • Feeling Weak
  • Fatigue
  • Muscle Twitching

Early Signs Can Be:

  • Tripping
  • Dropping Things
  • Slurred or 'Thick speech'
  • Muscle Cramping
  • Weight Loss

The earliest symptoms may include fasciculations, cramps, tight and stiff muscles (spasticity), muscle weakness affecting an arm or a leg, slurred and nasal speech, or difficulty chewing or swallowing. These general complaints then develop into more obvious weakness that may cause a physician to suspect ALS. The parts of the body showing early symptoms of ALS depend on which muscle in the body is affected. Many individuals first see the effects of the disease in a hand or arm as they experience difficulty with simple tasks requiring manual dexterity. In other cases, symptoms initially affect one of the legs and people experience difficulties when walking or running or they notice that they are tripping or stumbling more often. When symptoms begin in the arms or legs, it is referred to as “limb onset” ALS. Other individuals first notice speech problems, termed “bulbar onset” ALS. Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Individuals may develop problems with moving, swallowing (dysphagia) and speaking or forming words (dysarthria). Symptoms of upper motor neuron involvement include spasticity and exaggerated reflexes (hyperreflexia) including an overactive gag reflex. Symptoms of lower motor neuron degeneration include muscle weakness and atrophy, muscle cramps and fasciculations.

Diagnosis Edit

No one test can provide a definitive diagnosis of ALS. The diagnosis of ALS is primarily based on the symptoms and signs the physician observes in the patient and a series of test to rule out other diseases. Physicians obtain the patient's full medical history and usually conduct a neurological examination.

4. Physicians obtain the individual’s full medical history and usually conduct a neurologic examination at regular intervals to assess whether symptoms such as muscle weakness, atrophy of muscles, hyperreflexia, and spasticity are getting progressively worse.

To be diagnosed with ALS, people must have signs and symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes. One test cannot provide a definitive diagnosis of ALS. Instead, the diagnosis of ALS is primarily based on the signs and symptoms the physician observes in the patient and a series of tests to rule out other diseases. Physicians obtain the individual’s full medical history and usually conduct a neurologic examination to assess whether the patient’s symptoms are getting progressively worse. Since ALS symptoms in the early stages of the disease can be similar to those of a wide variety of other, more treatable diseases or disorders, appropriate tests must be conducted to exclude the possibility of other conditions. A few of these tests are Electromyography (EMG) a special recording technique that detects electrical activity in muscles, Nerve Conduction Study (NCS) which measures electrical energy by assessing the nerve’s ability to send a signal and Magnetic Resonance Imaging (MRI) a non-invasive procedure that uses a magnetic field and radio waves to take detailed images of the brain and spinal cord. Based on the person’s symptoms and findings from the examination and from these tests, the physician may order tests on blood and urine samples to eliminate the possibility of other diseases as well as routine laboratory tests. If some cases, for example, if a physician suspects that the individual may have a myopathy rather than ALS, a muscle biopsy may be performed. 

Treatment Edit

5. There is no cure for ALS; however, the Food and Drug Administration approved the first drug in 1995. The name of this Drug was riluzole and it believed that in order to reduce damage to motor neurons, you must decrease the release of glutamate. Clinical trials with ALS patients showed that riluzole prolongs survival by several months, mainly in those with difficulty swallowing.

There are no treatments for ALS that could slow or halt the progression of ALS, there are drugs or devices that can better control the symptoms and make living with the disease easier. Some of these drugs or devices include:

- Rilutek (riluzole): prevents the damage that can result from the nerve cell being overexcited by glutamate, improves functioning and survival

- Physical Therapy: improve circulation and help prolong muscle use in the early stages of ALS.

- Baclofen: relieves stiffness in in limbs and throat

- Phenytoin: eases cramps

- Tricyclic antidepressants: control excess saliva production

Strength testing

No cure has yet been found for ALS. However, the Food and Drug Administration (FDA) approved the first drug treatment for the disease, riluzole (Rilutek) in 1995. Riluzole is believed to reduce damage to motor neurons by decreasing the release of glutamate. Clinical trials with ALS patients showed that Riluzole prolongs survival by several months, mainly in those with difficulty swallowing. The drug also extends the time before an individual needs ventilation support. Riluzole does not reverse the damage already done to motor neurons, and people taking the drug must be monitored for liver damage and other possible side effects. Other treatments for ALS are designed to relieve symptoms and improve the quality of life for individuals with the disorder. This supportive care is best provided by multidisciplinary teams of health care professionals such as physicians; pharmacists; physical, occupational and speech therapists; nutritionists; social workers and home care hospice nurses. Working with patients and caregivers, these teams can design an individualized plan of medical and physical therapy and provide special equipment aimed at keeping patients as mobile and comfortable as possible. Physicians can prescribe medications to help reduce fatigue, ease muscle cramps, control spasticity and reduce excess saliva and phlegm. Medications are also available to help patients with pain, depression, sleep disturbances and constipation. Physical therapy and special equipment can enhance an individual’s independence and safety throughout the course of ALS. Gentle, low-impact aerobic exercise such as walking, swimming and stationary bicycling can strengthen unaffected muscles, improve cardiovascular health and help patients fight fatigue and depression. People with ALS who have difficulty speaking may benefit from working with a speech therapist. These health professionals can teach individuals adaptive strategies such as techniques to help them speak louder and more clearly. Nutritional support is an important part of the care of people with ALS. Individuals and caregivers can learn from speech therapists and nutritionists how to plan and prepare numerous small meals throughout the day that provide enough calories, fiber and fluid and how to avoid foods that are difficult to swallow.

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