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The motor neuron diseases 

The motor neuron diseases
Chapter:
The motor neuron diseases
Author(s):

Tom Jenkins

, Alice Brockington

, and Pamela J. Shaw

DOI:
10.1093/med/9780198746690.003.0604
Page of

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date: 05 March 2021

The motor neuron diseases can be considered an extended family of conditions with pathology affecting the lower and/or upper motor neurons, leading to clinical features of limb and/or bulbar weakness. Accurate diagnosis is essential to guide management, in particular, treatment options, any genetic implications, and prognosis. Amyotrophic lateral sclerosis is the family prototype and is a diffuse neurodegenerative disorder characterized by both upper and lower motor neuron cell death, causing progressive paralysis of limb, bulbar (speech and swallowing) and respiratory muscles, and resulting in death from respiratory failure. The cardinal clinical feature of combined upper and lower motor neuron signs in various body regions (bulbar, arms, trunk, legs) forms the basis for diagnostic criteria. There is no cure and the disease progresses relentlessly, with few patients surviving beyond 5 years from symptom onset.

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