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Specific Signs of ALS

Symptoms Involving Upper Motor Neurons

Motor neurons are nerve cells that help control muscle movement. Upper motor neurons carry messages from the brain to the spinal cord, where other cells then carry the message to the muscles, causing movement. ALS is a condition characterized by the degeneration or death of both upper and lower motor neurons.
 
Damage to the upper motor neurons can cause symptoms like tight and stiff muscles (spasticity) and exaggerated reflexes (hyperreflexia), including an overactive gag reflex. An abnormal reflex commonly referred to as Babinski's sign (the large toe extends upward as the sole of the foot is stimulated in a certain way) also indicates upper motor neuron damage.
 

Symptoms Involving Lower Motor Neurons

Lower motor neurons are found in the brainstem and spinal cord. This type of neuron relays messages from the upper motor neurons to the muscle itself. When lower motor neurons degenerate or die, the symptoms can include muscle weakness and atrophy, muscle cramps, and twitches that can be seen under the skin (fasciculations).
 
For a doctor to make an accurate ALS diagnosis, a person must have symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes.
 

Late ALS Symptoms

The pattern of when and where ALS symptoms first appear and how quickly the disease progresses can vary from person to person. But eventually, people who have ALS will not be able to:
 
  • Stand or walk
  • Get in or out of bed on their own
  • Use their hands and arms
  • Swallow or chew normally
  • Keep their body weight at a normal level.
     
Those with ALS symptoms are aware of their progressive loss of function because this disease generally does not affect a person's thinking or mental capacities. Therefore, people with this condition may experience anxiety and/or depression. Also, a small percentage of people may experience problems with their memory or decision-making abilities, and there is growing evidence that some people may develop a form of dementia.
 
In the later stages of ALS, the person will experience the following symptoms:
 
  • Difficulty breathing as the muscles of the respiratory system weaken
  • Loss of the ability to breathe on his or her own (must depend on ventilator support for survival)
  • An increased risk of pneumonia.
 
It's important for people with ALS (and their loved ones) to talk to their healthcare provider about the course of the disease and the options for treatment of ALS. This can help the patient make informed decisions in advance.
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