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ALS - Amyotrophic Lateral Sclerosis

DEFINITION - SYMPTOMS - TYPES - DIAGNOSIS - EXTREMITIES - BULBAR AND RESPIRATORY
PSYCHOLOGICAL - OTHER SYMPTOMS - SYMPTOM MANAGEMENT

DEFINITION:

Amyotrophic Lateral Sclerosis (ALS) is more commonly known as "Lou Gehrig's Disease". ALS is a progressive neuromuscular disease that causes degeneration of some of the largest of all nerve cells, called motor neurons. Motor neurons control the movement of voluntary muscles. Motor neurons extend from the brain to the spinal cord (the upper motor neuron)and from the spinal cord to the muscles throughout the body (the lower motor neurons). The disease causes the motor neurons to degenerate and eventually die. As the motor neurons die, the corresponding muscles are paralyzed. In the beginning of the disease, the person starts out with fatigue and becomes progressively weaker, eventually becoming paralyzed. Weakness and paralysis of the chest muscles and diaphragm caused by ALS lead to progressive respiratory insufficiency. In most cases, ALS results in fatality within two to five years from the first appearance of the symptoms. Problems with speech and swallowing may lead to loss of weight, and aspiration pneumonia.

Although ALS does not affect a person's mental capability, resulting conditions, stemming from respiratory insufficiency, can make the brain oxygen deprived. This oxygen deprivation causes symptoms like memory loss, incoordination, or personality changes.

The word Amyotrophic is derived from the Greek Language.

"A" means no or Negative.
"Myo" refers to Muscle.
"Trophic" means Nourishment.

Therefore, Amyotrophic means "no muscle nourishment ". The words "lateral sclerosis" identifies the areas in the spinal cord where portions of the nerve cells that activate the motor neurons and stimulate the muscles are located. As the disease advances, this area of the spinal cord undergoes a hardening or scaring ("sclerosis").

SYMPTOMS:

Approximately one third of patients become aware of the onset of the disease when their hands become clumsy, causing difficulty in the performance of fine tasks. Another one third experience weakness in the legs and may trip because of mild foot drop/flopping. The remaining one third notice slowing of speech or difficulty in swallowing. ALS may be present for some time before any symptoms are noticed. This lack of noticeable symptoms occurs because the lost or damaged nerve cells are compensated for by nerve cells that remain functioning. Early symptoms are generalized fatigue and fasciculations (involuntary twitching of muscle fibers) and cramps. As motor neurons deteriorate, patients may experience stiffness, cramps or occasional jerking of the arms or legs resulting from spasticity (muscle stiffness). Spontaneous tiny local areas of muscle twitching, called fasciculations are characteristic in most patients. Often symptoms begin in one area, such as the hands and feet, then spread to adjacent areas of the body. One side is usually more affected than others, in the early stages of the disease. Paralysis eventually may become virtually complete, except for the muscles of the eyes. Anal and bladder muscles and functions are not usually affected.

TYPES:

There are three main types of ALS:

Classic sporadic - In this frequent type, there is no known family history of the disease. This type accounts for 90 - 95% of ALS patients.
Familial - Infrequent type (5 - 10% of cases) is genetic or inherited and tends to affect younger individuals (about 50 years of age).
Mariana Island - This rare type occurs in Chamorro Indian patients in Guam.

DIAGNOSIS:

There is no specific clinical test that can identify ALS. Diagnosis is made by a neurologist through a physical examination, a thorough patient medical history, and neurological testing. Diagnostic testing often includes the electromyogram (EMG) to test muscle activity, nerve conduction velocity (NCV)to test nerve function, CT Scan, MRI (Magnetic Resonance Imaging), extensive blood work, and occasionally muscle biopsy.

The presence of signs of lower motor neuron disease (wasting and weakness of muscles and fasciculations), and upper motor neuron disease (spasticity, increased tendon reflexes, and abnormal reflexes like extensor plantar responses [Babinski reflexes]), without signs of involvement of other parts of the nervous symptom is used by a neurologist to make the diagnosis of ALS.