Amyotrophic Lateral Sclerosis

 

 

What is ALS?

Amyotrophic lateral sclerosis, also know as Lou Gehrig's disease, is a progressive neuromuscular disease that results in the loss of control over muscles and eventual death due to the inability to breath. Medical textbooks maintain that amyotrophic lateral sclerosis is almost always fatal, usually within two to five years of the diagnosis.

Amyotrophic Lateral Sclerosis is caused by destruction of brain and spinal cord nerve cells, with subsequent degeneration and atrophy of voluntary muscles. Depending on which muscles groups are affected, patients complain of weakness and loss of control in the arms, legs, swallowing, or talking. The mind is not affected, but eventually the respiratory muscles become weakened leading to impairment in breathing.

 

 

 

What are the signs and tests for ALS?

A neuromuscular examination indicates weakness, often beginning in one limb or in proximal groups (such as shoulders or hips). There may be muscle tremors, spasms, twitching, or muscle atrophy (loss of tissue). Atrophy and twitching of the tongue are common.

The gait may be stiff or clumsy. Reflexes may be abnormal, including loss of the gag reflex. Some patients have "emotional incontinence" in which it is hard to control crying or laughing. An EMG indicates that the motor nerves are not functioning, yet the sensory nerves are normal. A Head CT or MRI of head may be done to rule out other conditions. If there is a family history, a genetic test may be performed. A breathing test may be given to see if respiratory muscles are affected. Blood tests can exclude other conditions that may cause similar symptoms.

 

 

                    

 

Treatment for ALS

There is no known cure for ALS. Riluzole may prolong life, but does not reverse or stop disease progression. Treatment is aimed at controlling the symptoms. Baclofen or diazepam may be used to control spasticity that interferes with activities of daily living. Trihexyphenidyl or amitriptyline may be prescribed for people with impaired ability to swallow saliva. Physical therapy, rehabilitation, use of appliances (such as braces or a wheelchair) or orthopedic intervention may be required to maximize muscle function and general health.