Monday, February 8, 2021

History of Lou Gehrig’s disease

In the United States, ALS also is called Lou Gehrig’s disease, named for the Yankees baseball player who died of it in 1941. In Britain and elsewhere in the world, ALS is often called motor neuron disease in reference to the cells (motor neurons) that degenerate in this disorder.

Lou Gehrig signed with the New York Yankees in 1923 and in June 1925 began a streak of 2,130 consecutive games that ended only when he became weak 14 years later.

First described in 1869 by French clinician Jean-Martin Charcot, ALS is a misunderstood illness. Doctors once thought it was rare but now consider it fairly common: about 5,000 people in the U.S. are diagnosed with ALS every year.

The word “amyotrophic” comes from Greek roots that mean “without nourishment to muscles” and refers to the loss of signals nerve cells normally send to muscle cells. “Lateral” signifies the area of the spinal cord where portions of the dying nerve cells are located. “Sclerosis” means “hardened” and refers to the hardened nature of the spinal cord in advanced ALS.

The first description of multiple sclerosis dates back to the 14th century, but it was Jean-Martin Charcot and the use of the anatomoclinical method that made the first correlations between the clinical features of multiple sclerosis and the pathological changes noted post-mortem.

He described several cases of isolated progressive motor symptoms, with fasciculation, rigidity, contractures, bulbar involvement and death from respiratory failure. Charcot called this disease primary amyotrophic lateral sclerosis (ALS) and correctly identified the dysfunction of anterior horn cells as the pathology underlying the clinical features.

Charcot described and diagnosed the first cases of ALS as a specific neurological disease associated with a distinct pathology. Studies conducted between 1865 to 1869 by Charcot and his colleague Joffroy found that lesions within the lateral column in the spinal cord resulted in chronic progressive paralysis and contractures (no atrophy of muscles), while lesions of the anterior horn of the spinal cord resulted in paralysis without contractures (with atrophy of muscles).

Charcot's work on amyotrophic lateral sclerosis brought together neurological entities formerly considered as disparate disorders, primary amyotrophy and primary lateral sclerosis. In addition, these studies contributed to the understanding of spinal cord and brain stem anatomy and the organization of the normal nervous system.
History of Lou Gehrig’s disease

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