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5% of cases are hereditary

Starts in hands, feet or limbs

Healthy nerve cells are destroyed

No pain in early stages

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Neurological exams

“The lab report came back suggesting a neurological problem, and Morrie was brought in for yet another series of tests.  In one of those tests, he sat in a special seat as they zapped him with electrical current-an electric chair, of sorts- and studied his neurological responses” (Albom 7)

Blood and Urine tests

“He began to see doctors.  Lots of them.  They tested his blood. They tested his urine” (Albom 6)

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Although individuals experience symptoms differently, a common first response to feelings of muscular weakness, slurred speech or a change in ability to perform tasks such as buttoning clothes or tying shoes is to attribute these changes to "getting older."

Over time, however, an individual or a family member may realize that frequent tripping or falling, an inability to hold a full cup of coffee or slowed speech might be something more than a sign of aging. Occasionally, a coworker or boss may notice that the person's ability to perform at their normal capacity at work has changed. Rarely, a person may realize that he or she is having difficulty catching his or her breath while performing various tasks.

Clinicians Involved in the Diagnostic Process

An appointment with a primary care physician (PCP) is often the first step to understanding symptoms and changes. The time that elapses between first noticing symptoms to visiting the doctor varies widely among patients and often depends on how rapidly symptoms worsen.

A PCP typically refers an individual to a neurologist for further evaluation but this referral can be delayed if symptoms are minor or are developing and changing slowly. Consulting with other types of doctors is a common experience that many patients have. Once the symptoms are recognized as a neurological problem, a neurologist will be consulted.

A neurologist will perform a detailed physical examination that focuses on muscle strength, reflexes, coordination, and sensation. Typically, a neurologist will perform tests to rule out many of the other possible causes of a patient's symptoms. The type of symptoms, how fast or how slow they progress and change, and what other disease have to be excluded will affect the time it takes to determine the diagnosis.

Even after the tests rule out other disorders, a diagnosis of ALS may still be unclear. As a next step, individuals are often referred to a neuromuscular specialist, who specializes in nerves and muscles. The neuromuscular specialist will either assist in making a diagnosis or offer a second opinion to help confirm the possible diagnosis of ALS.

A neuromuscular specialist will review the results of the tests that have already been performed and may repeat some of them to see if there have been any changes over time. Meeting with a neuromuscular specialist will help ensure that the medical evaluation has been thorough and complete.

The Diagnostic Process

Step 1: Neurological Exam

The first important step in the diagnostic process is an examination by a neurologist. This will include detailed family, work, and environmental histories. During the exam, the neurologist will look for typical features of ALS that may include:

Muscle weakness (which is often only on one side of the body, such as one arm or one leg) as well as changes in the character of the individual's voice (especially slurred words or slowness of speech). The exam will evaluate muscles of the mouth, the tongue, and those involved in chewing and swallowing.

Lower Motor Neuron (LMN) features, such as muscles shrinking in size or muscle twitches. These twitches are called fasciculations and may occur when muscles contract without the nerve cells fully controlling them.

Upper Motor Neuron (UMN) features, such as hyperactive reflexes and muscle spasticity (a type of tightness and rigidity of the muscles).

Emotional changes resulting in the loss of some control of emotional responses, such as uncontrolled crying or laughing. The exam will also look at changes in thinking, such as loss of good judgment or loss of common social skills. The examiner will also look for problems in verbal fluency and word recognition abilities. These types of symptoms are less common or may be present but not readily noticeable.

The neurologist will also look for signs such as pain, loss of sensation, or extra-pyramidal rigidity, which is a different type of muscle rigidity that is frequently seen in Parkinson's type disorders.

Step 2: Diagnostic Tests

The next step in the diagnostic process often involves a series of test. These typically include an MRI (magnetic resonance imaging) of the neck, and sometimes of the head and lower spine, an EMG (electromyography) which tests nerve conduction, and a series of blood tests.

Sometimes urine tests, genetic tests, or a lumbar puncture (also called a spinal tap) are also necessary.
EMG
The EMG is a very important part of the diagnostic procedure. Although this test can sometimes be uncomfortable, it is very important to have it done.

In the first part of the EMG, small electric shocks are sent through the nerves to measure how fast they conduct electricity and to find out whether there is any nerve damage. The shocks tend to feel like the kind you get from static electricity but may sometimes feel a bit stronger.

This first part of the EMG determines whether the individual has "nerve block," which is a feature of a different disease called multifocal motor neuropathy. There is a chart at the end of this section which explains this disease a bit more. The first portion of the EMG also tests whether the nerves that communicate sensation are affected, which may also indicate a disease other than ALS.

The second part of the EMG tests the electrical activity of selected muscles. This is done by inserting a very fine needle into the selected muscles and using it to "listen" to the pattern of electrical activity in these muscles. No electric shocks are involved in this part of the test and the needle does not inject anything into, or take anything out of, your muscles.
Learn more about EMGs MRI
An MRI is a painless, non-invasive procedure that offers a very detailed picture of the spinal cord, the nerves that come out of the spinal cord, and the bones and connective tissues that surround and protect the spinal cord. It shows more detail than a CAT (computed axial tomography) scan or X-rays. The MRI will help rule out pressure on the spinal cord or major nerves (such as from a herniated vertebral disk), Multiple Sclerosis, and tumors or bony abnormalities that compress the nerves. It can help detect vascular changes and strokes that sometimes affect the spinal cord or brain.

The MRI takes about 30 minutes. The individual having the MRI lies down inside a machine that is basically a large, rotating magnet. The test is noisy but it is painless. Some people have trouble being in a small, confined spaces and it is important to tell the doctor about this before the MRI begins so that medication can be given to help the person relax.
Blood, Urine and Other Tests
Blood tests are used to look for evidence of other diseases whose symptoms are similar to early signs of ALS. These include tests for thyroid and parathyroid disease, vitamin B12 deficiency, HIV, hepatitis, auto-immune diseases, and some types of cancer. Creatine kinase (CK), a muscle enzyme released when muscles are injured or die, is also measured.

Specialized blood tests, such as autoimmune antibody tests, anti-GM1 antibody tests, and tests looking for high levels of protein in the blood and urine that may be related to some types of cancers, are also performed. Depending on the individual's work and environmental history, the doctor may also test his or her urine for heavy metals.

In some rare cases, genetic tests and tests of hexosaminidase A levels, which can be related to juvenile spinal muscle atrophy, may also be performed. Genetic testing for ALS is usually only done when someone else in the family has ALS.

Occasionally, a lumbar puncture (also called a spinal tap) may be required. For this test, a small needle is inserted into the lowest part of the spine (below the spinal cord) to remove fluid which will be examined for abnormal cells. A lumbar puncture is usually done only if the individual has unusual features of ALS, such as spinal nerve abnormalities, or has no sign of abnormal reflexes or spasticity. Similarly, some people who have uncommon patterns of weakness, pain, or very high creatine kinase (CK) levels may need a muscle biopsy to look for muscle-specific diseases. However, this is rarely necessary.

Step 3: Diagnosis

Once these tests have been completed, the neurologist may be able to tell whether an individual has ALS. Sometimes, not all of the symptoms and findings that are required to make the diagnosis are present, especially in the early phase of the disease. In this case, the neurologist will repeat the physical and neurological exams and the EMG at a later date to look for changes over time.
The symptoms and disorders chart below explains some of the alternative diseases that neurologists look for when trying to determine whether an individual has ALS. These disorders share some symptoms that resemble those seen in ALS. Modified from Layzer, RB. Chapter 415. Heredity and acquired intrinsic motor neuron diseases. In: Bennett and Plum (Eds), Cecil Textbook of medicine, W.B. Saunders, Philadelphia 1999.

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Diagnosing ALS: Symptoms, Clinicians & Tests

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Although individuals experience symptoms differently, a common first response to feelings of muscular weakness, slurred speech or a change in ability to perform tasks such as buttoning clothes or tying shoes is to attribute these changes to "getting older."

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40 to mid 60 are high risk

More men than women get it, however after 70 the percentages are the same

Smoking

Toxin exposure

Military

Some competitive sports

Use of herbicides when gardening

Head trauma and fractures

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Factors like head trauma or fracture, electric shock, playing some competitive sports, drinking water from private wells, use of herbicides for gardening, and taking specific dietary supplements all may be associated with the development of amyotrophic lateral sclerosis (ALS), according to a new study.

However, larger studies are required to confirm the findings, as only some of these factors reached statistical significance for their association with ALS.

The study, "Clinical and Lifestyle Factors and Risk of Amyotrophic Lateral Sclerosis: A Population-Based Case-Control Study," was published in the International Journal of Environmental Research and Public Health.

Despite progress in understanding the genetic causes of ALS, little still is known about the clinical and environmental risk factors predisposing an individual to the progressive neurological disease. To date, the only known risk factors include being male, of an older age, and having a family history of ALS.

While epidemiological studies have shown associations between ALS and a large number of environmental risk factors, evidence for each of them is insufficient and inconsistent, and none has been identified as a definite cause of the disease.

A group of Italian researchers now conducted a population-based study, targeting four provinces in both northern and southern Italy, to assess any possible environmental risk factors that may be contributing to the development of ALS.

They examined factors like clinical history - physical trauma, surgery, or blood donation - and lifestyle habits, focusing on food and alcohol intake, smoking status, source of water, and use of supplements. Leisure activities, including playing sports, gardening, and printing photographs, also were assessed.

The risk of ALS was estimated using statistical models adjusted for sex, age, and education level.

The study enrolled 230 participants, including 95 ALS patients and 135 healthy people (controls).

The results indicated that individuals who experienced physical trauma had a possible higher risk of ALS. Head trauma was associated with a 2.6-fold higher risk of developing ALS, while electric shock was linked with a 2.1-fold higher risk.

Further, playing certain sports at a competitive level, such as soccer, skiing, and swimming, is likely associated with increased risk for ALS.

The researchers also found a possible higher risk among people who reported using private wells for drinking water (1.38-fold) and for individuals who used herbicides during gardening (1.95-fold).

Consuming some dietary supplements, particularly those that contain amino acids, also showed a potential association with increased ALS risk. In the southern Italian population, vitamins and minerals such as selenium also were likely associated with a higher ALS risk.

Conversely, people who ate fish three times a week or more had a 73% lower risk of developing ALS.

However, the association between fish consumption and ALS was considerably weaker in women compared with men, and there was no evidence of a dose-response relation, meaning that eating fish more than three times per week was not associated with a decreased risk.

No differences in ALS risk were found in relation to alcohol consumption, according to the researchers. They did, however, find a possible association between ALS and smoking, which is consistent with previous studies.

"The results of the present case-control study suggest a possible association between some non-genetic risk factors and ALS risk, namely trauma, particularly head trauma or fracture, electric shock, some competitive sport activities, use of private wells for drinking water, use of herbicides during gardening and consumption of some specific dietary supplements," the researchers said.

However, while many potential factors were identified in this study, only head trauma and fish consumption showed significant associations with ALS risk, the team said. This suggests that more studies, with larger populations, are needed to investigate the additional risk factors.

Iqra holds a MSc in Cellular and Molecular Medicine from the University of Ottawa in Ottawa, Canada. She also holds a BSc in Life Sciences from Queen's University in Kingston, Canada. Currently, she is completing a PhD in Laboratory Medicine and Pathobiology from the University of Toronto in Toronto, Canada. Her research has ranged from across various disease areas including Alzheimer's disease, myelodysplastic syndrome, bleeding disorders and rare pediatric brain tumors.

Inês holds a PhD in Biomedical Sciences from the University of Lisbon, Portugal, where she specialized in blood vessel biology, blood stem cells, and cancer. Before that, she studied Cell and Molecular Biology at Universidade Nova de Lisboa and worked as a research fellow at Faculdade de Ciências e Tecnologias and Instituto Gulbenkian de Ciência. Inês currently works as a Managing Science Editor, striving to deliver the latest scientific advances to patient communities in a clear and accurate manner.

Iqra holds a MSc in Cellular and Molecular Medicine from the University of Ottawa in Ottawa, Canada. She also holds a BSc in Life Sciences from Queen's University in Kingston, Canada. Currently, she is completing a PhD in Laboratory Medicine and Pathobiology from the University of Toronto in Toronto, Canada. Her research has ranged from across various disease areas including Alzheimer's disease, myelodysplastic syndrome, bleeding disorders and rare pediatric brain tumors.

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Trauma, Sports, Herbicides, Other Factors May Increase ALS Risk...

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Factors like head trauma or fracture, electric shock, playing some competitive sports, drinking water from private wells, use of herbicides for gardening, and taking specific dietary supplements all may be associated with the development of amyotrophic lateral sclerosis (ALS), according to a new...

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Begins with muscle twitching or weakness in a limb or slurred speech

Difficulty walking

Tripping and falling

“He kept tripping, so he purchased a cane.” (Albom 8)

Trouble swallowing

Inappropriate emotions (laughing, crying or yawning)

Muscle cramps, twitching in the arms, shoulders and tongue

Coughing

“He closed his eyes, then coughed.  Then he coughed again.  Then he coughed again more loudly.” (Albom 105)

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Lung failure

“ ‘I’m going to suffocate. Yes. My lungs, because of my asthma, can’t handle the disease.’ “(Albom 36)

Dementia

Pressure sores

Pneumonia

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Arms and hands become numb

Daily tasks become difficult

Some may experience the following: fatigue, poor balance, slurred speech, loss of grip strength, tripping or falling, cramping and twitching

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All voluntary muscle movements are paralyzed

Muscles that are involved in breathing, eating and speaking are paralyzed

Eating is made possible by a feeding tube

Breathing is made possible by a ventilator

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Amyotrophic lateral sclerosis (ALS) is a progressive neurological disease characterized by the destruction of nerve cells (neurons) that are responsible for controlling voluntary muscle movement. Examples of voluntary muscle movement include chewing, walking, talking and breathing.

Patients may not experience the same symptoms and, for some, the disease progresses more slowly than others. ALS cannot be cured, but treatments do exist.

In general, ALS progression may be divided into three distinct stages: early, middle, and late. The following section offers information about the characteristic symptoms associated with each stage.

Early stage ALS

Early symptoms of ALS are usually characterized by muscle weakness, tightness (spasticity), cramping, or twitching (fasciculations). This stage is also associated with muscle loss or atrophy.

Symptoms at this stage are generally limited to a particular part of the body, and often first begin in the arms or hands, making daily tasks, such as buttoning clothes or opening a can of food, difficult to execute. Alternatively, they may first appear in a leg - in either case, disease that begins in the arms or legs is often called "limb onset" ALS. The muscular issues may cause people to experience fatigue, poor balance, slurred words, loss of grip strength, or to trip or fall when walking.

Middle stage ALS

During the middle stages of ALS, muscle weakness and atrophy spreads to other parts of the body. Some muscles become paralyzed, while others lose strength. Some infrequently used muscles may become permanently shortened, causing contractures in which joints (e.g., elbows) are no longer able to fully straighten.

People may also develop problems in walking, and in swallowing (dysphagia) and chewing food, which increases the risk of choking. Other problems associated with increasing muscle weakness include difficulty in speaking and breathing (dyspnea), and possibly uncontrollable and inappropriate laughing or crying, a reaction called the pseudobulbar affect.

Most ALS patients retain higher mental and reasoning abilities, and are aware of their progressive loss of muscular function.

Late stage ALS

As ALS progresses, most voluntary muscles become paralyzed. As the muscles of the mouth and throat, and those involved in breathing, become paralyzed, eating, speaking, and breathing is compromised. During this stage, eating and drinking are usually require a feeding tube. Breathing is assisted via a ventilator.

Most people with ALS die due to respiratory failure, and the prognosis is usually three to five years after the first symptoms appear. Less common causes of death may result from malnutrition, pulmonary embolism (obstruction of a lung artery), heart arrhythmias(the heart beating faster or slower than normal, or irregularly), and pneumonia due to aspiration (which occurs when food or water gets into the lungs).
Note: ALS News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

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Amyotrophic lateral sclerosis (ALS) is a progressive neurological disease characterized by the destruction of nerve cells (neurons) that are responsible for controlling voluntary muscle movement. Examples of voluntary muscle movement include chewing, walking, talking and breathing. Patients may not experience the same symptoms and, for some, the disease progresses more slowly than others.

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Muscle weakness spreads further into the body

Some muscles become fully paralyzed

Some joints (elbows and knees) are no longer able to fully straighten

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Although Mitch doesn’t cry until Morrie dies, we see him think about Morrie’s positive attitude and constant ability to stay happy.

“He smiled. “Not everyone is so lucky.” I studied him in his chair, unable to stand, to wash, to pull on his pants. Lucky? Did he really say lucky?” (Albom 57)

Mitch thinks this while Morrie is talking about giving himself self-pity in the mornings.  He cry’s a few tears but shuts it off after he feels around his body to see how limp he is.

“ ‘Mitch, I don’t allow myself any more self-pity than that. A little each morning, a few tears, and that’s all.’ “(Albom 57)

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Ask first if they need assistance

Don’t try and take over tasks they can still do on their own

Patience is key

Let the individual do the tasks they can do, even if it takes them longer than a person without ALS

Use adaptive devices

a computer for paying bills, a mechanical can opener, a door helper, easier devices to help with unbuttoning shirts and unzipping jackets, give them a stool to sit in when showering

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Medications

Rilutek: reduces damage to motor neurons

Radicava: slows the decline of muscle function and ability to do everyday tasks

Physical therapy can help as well