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© Cambridge University Press 2011. Introduction Motor neuron disease (MND) refers to a family of related progressive disorders in which upper and lower motor neurons cease to function, thus causing increasing disability. A French neurologist, Jean-Martin Charcot, first suggested grouping together disparate conditions that affect the lateral horn of the spinal cord as MND in 1869. The disease came to public awareness in 1939, when the New York Yankees baseball icon Lou Gehrig was diagnosed with the disease and died a few years later at the age of 37. Despite the relative rarity of the disease, a number of high-profile cases, including the actor David Niven and Cambridge Professor of Mathematics Stephen Hawking, have kept the disease in the public arena. Although the symptoms of the various forms of MND vary at the outset, they overlap in later stages. Amyotrophic lateral sclerosis (ALS) is the most common type, accounting for approximately 8 of 10 cases. Initial symptoms are typically stiffness and weakness in the hands and feet. ALS is a fatal condition, with people living between 2 and 5 years from initial symptoms. Almost all other cases of MND are progressive bulbar palsy (PBP), with initial bulbar involvement, causing difficulties with speech, chewing, and swallowing. Once again, this disease is fatal with the majority of people with the condition dying within 3 years from presentation of the first symptoms. Two other forms of MND have been documented but are very rare. Progressive muscular atrophy begins with weakness, but not stiffness, in the hands and feet.

Original publication

DOI

10.1017/CBO9780511975363.005

Type

Chapter

Book title

Quality of Life Measurement in Neurodegenerative and Related Conditions

Publication Date

01/01/2011

Pages

41 - 51