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What Is Affected First? | Progression
It is important to realize that in some diseases, obvious symptoms could be exhibited early on. In ALS, this is not the case, making this neuromuscular disease even more deadly. By the time there are any signs suggesting possible problems, 50%-80% of motor neurons in a muscle may already have died. The following are some of the earliest symptoms shown in people with ALS:
Since symptoms could be as minor as trouble buttoning a shirt or turning a key, they might be overlooked at first, letting the disease progress unknown to the person, leaving the body diminished in strength and in its ability to control voluntary movement. Signs of upper motor neuron damage usually include fasciculation, spasticity, clonuses, and exaggerated reflexes, while signs of lower motor neuron damage is shown as muscle weakness and atrophy.
Different people will often exhibit initial symptoms in different areas of the body. When the first symptoms occur in the arms or legs, this is called Limb-Onset ALS, and accounts for about 68% of all cases, 32% and 36% respectively. Difficulties in swallowing or speaking as initial symptoms is termed Bulbar-Onset ALS, referring to the bulbar region, an area in the brain stem, being affected; Bulbar-onset ALS is seen in about 25% of all cases. Less common is Dyspnea, or trouble breathing, which shows up in only 7% of total cases.
Those with limb-onset ALS will gradually experience muscle weakness throughout their body, losing their capabilities to chew, swallow, and speak as well, as the disease progresses and the muscles of the mouth and throat weaken. Those experiencing bulbar-onset ALS will lose function later on in their arms and legs. In the latest stages of the disease, the results are the same--a person weakened, wasted, and paralyzed, who might not be able to walk, move, or even speak, but having intact bowel, bladder, sexual, cardiac, and visual functions. Besides those, intelligence and memory are not affected, leaving those with ALS to be fully aware of what is happening to their body.
The progression of ALS can last as short as six months or as long as years. Very rarely, a person's symptoms may level out or stop; other times there will be a reversal for unknown reasons, but for the most part, 50% of ALS-afflicted people die within three years, 30% will live for three to five years, and 20% will live more than five years. Of this last 20%, only half will live to see another five or more years.