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Amyotrophic Lateral Sclerosis

1

Amyotrophic lateral sclerosis, also known as Lou Gehrig's disease and motor neurone disease, is a specific disease that causes the death of neurons which control voluntary muscles.

A motor neuron is a nerve cell whose cell body is located in the spinal cord and whose fiber projects outside the spinal cord to directly or indirectly control effector organs, mainly muscles and glands.

Henry Louis "Lou" or "Buster" Gehrig was an American baseball first baseman who played 17 seasons in Major League Baseball for the New York Yankees, from 1923 through 1939.

Skeletal muscle is one of three major muscle types, the others being cardiac muscle and smooth muscle.

Amyotrophic Lateral Sclerosis - ALS by EmpoweRN

2

Some also use the term "motor neuron disease" for a group of conditions of which ALS is the most common.

A motor neuron disease is any of several neurological disorders that selectively affect motor neurons, the cells that control voluntary muscles of the body.

ALS - Amyotrophic Lateral Sclerosis - Lou Gehrig's Disease by robertmorsend

3

ALS is characterized by stiff muscles, muscle twitching, and gradually worsening weakness due to muscles decreasing in size.

4

This results in difficulty in speaking, swallowing, and eventually breathing.

5

The cause is not known in 90% to 95% of cases.

6

About 5–10% of cases are inherited from a person's parents.

7

About half of these genetic cases are due to one of two specific genes.

A gene is a locus of DNA which is made up of nucleotides and is the molecular unit of heredity.

8

The diagnosis is based on a person's signs and symptoms with testing done to rule out other potential causes.

9

No cure for ALS is known.

10

A medication called riluzole may extend life by about two to three months.

11

Non-invasive ventilation may result in both improved quality and length of life.

Mechanical ventilation is the medical term for artificial ventilation where mechanical means is used to assist or replace spontaneous breathing.

12

The disease usually starts around the age of 60 and in inherited cases around the age of 50.

13

The average survival from onset to death is two to four years.

14

About 10% survive longer than 10 years.

15

Most die from respiratory failure.

16

In much of the world, rates of ALS are unknown.

17

In the United States the disease affects about four people per 100,000 per year.

18

Descriptions of the disease date back to at least 1824 by Charles Bell.

Sir Charles Bell KH FRS FRSE FRCSE MWS was a Scottish surgeon, anatomist, neurologist, and philosophical theologian.

19

In 1869, the connection between the symptoms and the underlying neurological problems was first described by Jean-Martin Charcot, who in 1874 began using the term amyotrophic lateral sclerosis.

Jean-Martin Charcot was a French neurologist and professor of anatomical pathology.

20

It became well known in the United States in the 20th century when in 1939 it affected the baseball player Lou Gehrig and later worldwide when physicist Stephen Hawking, diagnosed in 1963 and expected to die within two years, became famous.

Stephen William Hawking, CH, CBE, FRS, FRSA is an English theoretical physicist, cosmologist, author and Director of Research at the Centre for Theoretical Cosmology within the University of Cambridge.

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