What are the other Names for this Condition? (Also known as/Synonyms)

• Bulbar Motor Neuron Disease
• Charcot's Syndrome
• Dementia with Amyotrophic Lateral Sclerosis

What is Amyotrophic Lateral Sclerosis? (Definition/Background Information)

• Amyotrophic Lateral Sclerosis (ALS) is a disease of the nerve cells (neurons) in the brain and spinal cord that controls voluntary muscle movement. This progressive disorder is also known as Lou Gehrig's Disease
• ALS generally affects adult men over the age of 40 years. The condition may be inherited in some cases, but largely the cause of Amyotrophic Lateral Sclerosis remains unknown
• Since, most muscles of the body are affected, individuals with ALS lose control over their muscles leading to signs and symptoms, such as difficulty performing simple physical tasks, speech and swallowing problems, dementia, and even respiratory failure
• A combination of medications, physical therapy and rehabilitation, are typically used to treat Amyotrophic Lateral Sclerosis and reduce the progression of this rapidly deteriorating disorder
• However, the prognosis for ALS is poor, and the survival period is only about 3-8 years, after onset of the signs and symptoms 

Who gets Amyotrophic Lateral Sclerosis? (Age and Sex Distribution)

• Amyotrophic Lateral Sclerosis affects adult individuals between the age of 40 and 70 years. However, the condition may occur at any age
• Men are affected more commonly than women
• Individuals of all races and ethnic groups can be affected

What are the Risk Factors for Amyotrophic Lateral Sclerosis? (Predisposing Factors)

The risk factors of Amyotrophic Lateral Sclerosis could include:

• Having a family member with the disease
• Head injury
• Individuals in military service; the cause of increased incidence in military personnel is unknown
• Participation in certain contact sports
• Advancing age
• Smoking
• Exposure to lead
• Certain viral infections

 It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider. 

What are the Causes of Amyotrophic Lateral Sclerosis? (Etiology)

• The cause of Amyotrophic Lateral Sclerosis is unknown in a large majority of cases
• In ALS, the nerve cells (neurons) are unable to repair themselves and can no longer send signal to the muscles, which results in a breakdown in communication, between the muscles and the brain
• This progressively and eventually leads to a weakening of the muscles, which are then unable to move the arms, legs, and body
• In rare cases, Amyotrophic Lateral Sclerosis may be inherited. It is estimated that 5 cases in 100 cases of ALS may be hereditary
• Chemical imbalance in the brain
• Dysfunctional immune system 

What are the Signs and Symptoms of Amyotrophic Lateral Sclerosis?

The signs and symptoms of Amyotrophic Lateral Sclerosis include:

• Weakness or difficulty in moving the body, leading to difficulty performing simple tasks, such as lifting, climbing stairs, and walking
• ‘Head drop’ due to weakness of the neck muscles
• Changes in speaking or swallowing
• Voice changes, like hoarseness of voice
• Unusual muscle twitches, spasms, or cramps
• Unusual weight loss or loss of muscle bulk
• Difficulty controlling one’s cry or laughter
• Difficulty in breathing
• Speech difficulties may include slurring of speech
• Dementia causing memory problems
• Signs and symptoms of lung infection 

How is Amyotrophic Lateral Sclerosis Diagnosed?

A diagnosis of Amyotrophic Lateral Sclerosis may be made through the following tests and exams:

• Complete physical examination with a thorough evaluation of family and personal medical history. The physician will test for muscle reflexes, muscle tone, balance, and coordination
• Breathing tests, to see if the chest muscles are affected
• Cervical spine CT or MRI scans to ensure that there is no disease or injury to the neck
• Electromyography (EMG) to examine which nerves or muscles do not work properly, and nerve conduction studies
• Genetic testing, if there is a family member with the disease
• Blood tests to rule out other conditions or infections
• Head CT or MRI of the brain to rule out other conditions
• Swallowing studies
• Lumbar puncture studies (examination of the cerebrospinal fluid surrounding the brain); also known as a spinal tap
• Muscle biopsy to rule out other conditions or disorders - the specimen is examined under a microscope by a pathologist, to arrive at a definitive diagnosis 

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis. 

What are the possible Complications of Amyotrophic Lateral Sclerosis?

The following complications may occur with Amyotrophic Lateral Sclerosis disorder:

• Memory loss (dementia)
• Difficulty in swallowing, leading to malnutrition, aspiration, and pneumonia (lung infection)
• Difficulty in breathing, necessitating a tracheostomy; a procedure in which a hole is created in front of the neck to help with breathing
• Weight loss

Respiratory failure is the most common cause of death in individuals with ALS. 

How is Amyotrophic Lateral Sclerosis Treated?

The following treatment options are available for individuals diagnosed with Amyotrophic Lateral Sclerosis:

• Riluzole helps to slow down the symptoms, thus helping improve the quality of one’s life
• Baclofen or diazepam may be used to decrease the stiffness of the muscles that interferes with the performance of one’s daily activities
• Trihexyphenidyl or amitriptyline may be used to help with swallowing difficulties
• Physical therapy and rehabilitation, use of braces, wheelchairs, or other assistive devices, are essential to help with impaired muscle function
• Speech therapy to help with speech difficulties
• Breathing devices are recommended to ease some of the breathing problems. A tracheostomy may be performed in case the individual is facing acute breathing problem
• Sometimes, a tube is placed into the stomach for feeding, by a procedure called a gastrostomy
• Psychological, social, and family support is very critical

How can Amyotrophic Lateral Sclerosis be Prevented?

Currently, there is no established or known method to prevent Amyotrophic Lateral Sclerosis, or to alter its course. 

What is the Prognosis of Amyotrophic Lateral Sclerosis? (Outcomes/Resolutions)

• The prognosis of Amyotrophic Lateral Sclerosis is generally dismal, even with medical intervention (treatment and therapy), as it is a rapidly-degenerative disorder
• The condition usually progresses very rapidly and leads to death from respiratory infection, within 3-5 years
• About 10% of individuals with ALS live longer than 8 years 

Additional and Relevant Useful Information for Amyotrophic Lateral Sclerosis:

There are voluntary organizations and support groups that provide help and understanding to the individuals, families and friends of the Amyotrophic Lateral Sclerosis affected.