Motor neuron disease (ALS)

Legs | Rheumatology | Motor neuron disease (ALS) (Disease)


Description

Amyotrophic lateral sclerosis (ALS), also referred to as motor neuron disease, is the most common form of the motor neuron diseases. The disorder is characterized by rapidly progressive weakness, muscle atrophy and fasciculations, muscle spasticity, difficulty speaking (dysarthria), difficulty swallowing (dysphagia), and decline in breathing ability.

Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrigs disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles. The disease belongs to a group of disorders known as motor neuron diseases, which are characterized by the gradual degeneration and death of motor neurons.

In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away (atrophy), and twhich (fasciculations) . Eventually, the ability of the brain to start and control voluntary movement is lost.

ALS causes weakness with a wide range of disabilities (see section titled What are the symptoms?). Eventually, all muscles under voluntary control are affected, and patients lose their strength and the ability to move their arms, legs, and body. Although the disease usually does not impair a persons mind or intelligence, several recent studies suggest that some ALS patients may have alterations in cognitive functions such as depression and problems with decision-making and memory.

ALS does not affect a persons ability to see, smell, taste, hear, or recognize touch. Patients usually maintain control of eye muscles and bladder and bowel functions, although in the late stages of the disease most patients will need help getting to and from the bathroom.

Causes and Risk factors

In ALS, the nerve cells that control the movement of the muscles gradually die, so the muscles progressively weaken and begin to waste away. Up to 1 in 10 cases of ALS is inherited. But the remainder appear to occur randomly. It may be that ALS, similar to other diseases, is triggered by certain environmental factors in people who already carry a genetic predisposition to the disease. For example, some studies examining the entire human genome (genome-wide association studies) found numerous genetic variations that people with ALS held in common, and that might make a person more susceptible to ALS.

Diagnosis and Treatment

Amyotrophic lateral sclerosis is difficult to diagnose early because it may appear similar to several other neurological diseases. Because theres no reversing the course of amyotrophic lateral sclerosis, treatments mainly involve efforts to slow the progression of symptoms and make you more comfortable and independent. ...