AMYOTROPHIC LATERAL SCLEROSIS

BAsic information

description

A progressive breakdown of the cells of the spinal cord. This results in a gradual loss of muscle function. It involves the central nervous system and the muscle system, especially in the hands, forearms, legs, head, and neck. It usually affects people aged 40 to 60 years, and occurs more often in men than women.

frequent signs and symptoms

• Muscle twitching and weakness. It begins in the hands and spreads to the arms and legs. The weakness then begins to affect muscles that control breathing and swallowing.
• Muscle cramps.
• Stiffening and spasticity of muscle groups.
• Weight loss.
• Slurring of speech.
• Mental function is rarely affected.
• Sudden involuntary bursts of laughter or crying.

causes

Unknown. Research suggests that there may be more than one cause.

risk increases with

• Age over 40.
• Family history of ALS.
• Smoking.

preventive measures

Cannot be prevented at present.

expected outcomes

• This condition is currently considered incurable. It is usually fatal in 2 to 5 years, but 20% of patients survive 5 years and 10% survive 10 years.
• Medical research into causes and treatment continues. There is hope for more effective treatment.

POSsible complications

• The disorder affects the patient's personal relationships, career, income, muscle coordination, sexuality, and energy.
• Progressive inability to walk and to do things involved with daily living, such as being able to feed oneself.
• Wheelchair use is needed.
• Pressure sores or skin infections due to being bedridden or in a wheelchair.
• Pneumonia due to swallowing difficulty and choking.
• The disorder is eventually fatal due to respiratory muscle weakness.

diagnosis & treatment

general measures

• Your health care provider will do a physical exam and ask questions about your symptoms. Medical tests will include nerve studies (electromyography and nerve conduction velocity). Blood and urine studies, x-rays, and other tests may be done. No one test diagnoses ALS.
• There is no specific treatment. Supportive care is provided to relieve symptoms and for complications.
• Aids for helping with daily living are available. These can help maintain some function and quality of life.
• Counseling may be helpful in finding ways for both the patient and the family to cope with the diagnosis.
• Surgery for tracheostomy is usually required once breathing difficulties develop.
• In later stages of the disease, the patient will require complete nursing care.
• Patient and family may benefit from hospice care.
• To learn more: ALS Association, 27001 Agoura Rd., Suite 150, Calabasas Hills, CA 91301; (800) 782-4747; website: www.alsa.org.

medications

• Riluzole may be prescribed. It can help delay the progress of ALS for a few months.
• Antibiotics will be prescribed if infection develops.
• Baclofen or tizanidine may help reduce spasticity.
• Antidepressant may help to decrease excess saliva.

activity

• Stay as active as possible. Weakness will gradually limit movement. A physical therapy program can help to maintain independence as long as possible.
• Obtain equipment that will aid in mobility, such as a walker or wheelchair.

diet

• Soft, easy-to-swallow foods may be needed if swallowing is a problem.
• May require tube feedings eventually.

notify our office if

• You or a family member has symptoms of amyotrophic lateral sclerosis.
• After diagnosis, symptoms occur that cause concern.