Amyotrophic Lateral Sclerosis

General Illness Information

Common Name:
AMYOTROPHIC LATERAL SCLEROSIS
(Lou Gehrig’s Disease)

Medical Term: None specified

Description: A progressive disease which usually begins with muscle weakness most frequently in the hands and less often in the feet .Weakness is usually asymmetric (usually progresses more on one side of the body) and is accompanied by muscle wasting and proceeds either up the arm or down the leg. It is due to progressive breakdown of the cells of the spinal cord, resulting in gradual loss of muscle function. This is not contagious or cancerous. It involves the central nervous system and affects the muscle system, especially in the hands, forearms, legs, head and neck. Median age of onset is age 55. Affects males more than females. 5% of the cases are familial.

Causes: Usually unknown.

However, in 5-10% of the cases there is a familial tendency with an autosomal dominant inheritance tendency.

Prevention: Cannot be prevented at present.

Signs & Symptoms

  • Muscle twitching (fasciculation) and weakness, beginning in the hands and spreading to the arms and legs. Weakness eventually affects muscles that control breathing and swallowing.
  • Muscle cramps
  • Stiffening and spasticity of muscle groups
  • Unexplained weight loss
  • Slurring of speech
  • Difficulty swallowing
  • Mental deterioration usually does not occur
  • Sensory systems and urinary sphincters are spared

Risk Factors

  • Age over 40 years
  • Family history of ALS

Diagnosis & Treatment

General Measures:

  • Diagnosis is usually made from history and physical examination. Physicians usually suspect ALS when a middle aged patient develops muscle weakness without loss of sensation. Physical examination and tests will help rule out other causes of weakness.
  • Diagnostic testing will include electromyography to measure nerve conduction. This is the most useful test in diagnosing ALS.
  • There is no specific treatment. Supportive care is provided to control symptoms and for complicating emergencies
  • Obtain good nursing care to prevent pressure sores (caused by immobility).
  • Learn to do self-suction in order to handle increased accumulation of secretions in the lungs.
  • Counseling to learn to cope with disability.
  • Eventual hospitalization or nursing-home care.
  • Patients may benefit from a hospice program or local chapter of the ALS support group. Information available from ALS Association, 21021 Ventura Blvd., Woodland Hills, CA 91364, (800)782-4747

Medications:

  • There are no medications to treat ALS.
  • Antibiotics to fight infection if pneumonia develops.
  • Antidepressant may help to decrease saliva production.
  • Medications may be prescribed to help reduce spasticity.

Activity:

  • Medications may be prescribed to help reduce spasticity.
  • Obtain equipment that will aid in mobility, such as walker or wheelchair.

Diet:

  • If swallowing is difficult, then try to eat soft, easy-to-swallow foods.
  • May require tube feedings occasionally.

Possible Complications :

  • Pressure sores caused by immobility
  • Pneumonia caused by swallowing difficulty and choking
  • The progressive physical degeneration affects the patient’s relationships, career, income, muscle coordination, sexuality and energy.

Prognosis

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.

Scientific research into causes and treatment continues so there is hope for increasingly effective treatment and cure.