History of ALS

Jean- Marie Charcot (1825-1893) noted the first reports of the characteristics of ALS in 1874, and named the fatal syndrome based on what he found.  He was a noted French neurologist who has been called “the Father of Neurology”, and explained how the central nervous system works. 

Damage to upper motor neurons, which begin at the top of the brain, results in muscle weakness, stiffness, and augmented reflexes.  Lower motor neurons start at the base of the brain and spinal cord; injury to these neurons causes muscle atrophy, twitching, weakness, and reduced reflexes.  When motor neurons in the brain are affected, muscles for talking, chewing, and swallowing weaken.  When lower spinal cord motor neurons are involved, movement is lost in the limbs, neck, and trunk.

ALS weakens skeletal (voluntary) muscles. It is usually considered painless.  The body is ultimately fully paralyzed; patients must use respirators to breathe. For some, ALS affects the limbs first, making it hard to move; this is called limb- onset ALS.  In others, the symptoms affect the facial muscles, speech, and swallowing. This condition is called bulbar- onset ALS.  Many have muscle spasms, cramps, and atrophy.  Since ALS is a progressive condition, most will suffer from all of these symptoms.

In the US, approximately 5,000 people are diagnosed yearly with ALS.  Most are between the ages of 45-65, although many are not.  Presently in the United States, about 30,000 people are victims of ALS; most will die within 3-5 years of contracting the fatal syndrome.  Ten percent of all known cases are hereditary.  Although males are more prone to ALS, the condition has no racial or ethnic boundaries. 

Drs. Scarmeas, Shih, Stern, Ottoman, and Rowland recently published a scientific article, concluding that subjects with motor neuron diseases were more likely to be slim, or had once been serious athletes. Many famous people in US history have had ALS. For example, Lou Gehrig was a very famous Yankee baseball player and Ezzard Charles was a heavyweight-boxing champion. We found there were many other such people, such as Catfish Hunter, another baseball player, and many football players as well.  Senator Jacob Javits was an avid tennis player.  David Niven was a competitive sailor.  Even some in the musical field were subjected to ALS; Dimitri Shostakovitch and Charles Mingus both vigorously practiced musical instruments. Stephen Hawking, the famous physicist, has ALS as well.