1 1 What Is ALS? It starts with muscle weakness in the arms, hands, legs, or feet that often is ignored by the individual. Instead of going away, the weakness becomes more profound over a period of weeks to months and expands from an initially small region to the whole limb. Some people develop stiffness or muscle twitches (fasciculations) as the control of muscles in the face and neck becomes compromised, spreading beyond to muscles that regulate swallowing, chewing, and speaking and that also control facial expressions. They suffer excessively strong reflex responses with- out apparent cause. Virtually all voluntary muscles, muscles that require the brain to send them a signal to contract, are susceptible to the inexo- rable progression of the symptoms. Involuntary muscle types, like the heart muscle, which does not require brain activation to beat, are unaffected in ALS. Certain voluntary muscles, such as those governing sphincters and eye movement, for some unknown reason remain unaffected. As ALS progresses, the weakness that began in an extremity or on one side of the body becomes more generalized, spreading to larger and larger areas and eventually affecting both left and right sides of the body. The muscles of the diaphragm that control breathing become weaker and weaker, leading to paralysis of the diaphragm and death in the majority of ALS patients. Breathing difficulties combined with head and neck muscu- lar weakness make it difficult to swallow and result in increasing instances
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