Motor neurone disease sclerosis

AMYOTROPHIC LATERAL SCLEROSIS IS THE MOST COMMON ADULT ONSET MOTOR NEURON DISEASE 731... 

The motor neuron diseases (MND), including amyotrophic lateral sclerosis (ALS), are chronic, progressive illnesses characterized by severely disabling clinical features... 

Cork, L. C., Griffin, I. W., Adams, R. J. and Price, D. L. Motor neuron disease spinal muscular atrophy and amyotrophic lateral sclerosis. Animal model hereditary canine spinal muscular atrophy. Am. J. Pathol. 100 599-602,1980. 

One group of people who may be at greater risk are those who are exposed to cyanide but are unable to smell the chemical (Kirk and Stenhouse 1953 Snodgrass 1996). Patients with motor neuron disease (amyotrophic lateral sclerosis) possess a disorder in cyanide detoxification that may result in their higher susceptibility to cyanide (Kato et al. 1985). 

Motor neurone disease is often called amyotrophic lateral sclerosis in the USA, where it is also known as Lou Gehrig s disease after the famous New York Yankees baseball player who developed the disease in 1939. 

Key words Motor neuron diseases, amyotrophic lateral sclerosis (ALS), spinal muscular atrophy (SMA), peripheral neuropathies, Charcot-Marie-Tooth diseases, hereditary motor and/or sensory neuropathies (HSMNs), congenital myasthenic syndromes, neuromuscular junction, muscular dystrophies, Duchenne s disease. 

The disease models can be grouped into four primary categories (Fig. 20.1). (1) Motor neuron diseases, in which the death of motor neuron somata in the spinal cord results in denervation of the muscles, progressive flaccid paralysis, and usually premature death. In humans, examples of such diseases would include amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). (2) Peripheral neuropathies, in which axonal integrity or conduction is not maintained, resulting in axon degeneration and impaired connectivity of the nervous system and the musculature. 

Amyotrophic lateral sclerosis (ALS) In UK, known as motor neuron disease. A devastating adult onset paralytic disorder caused by degeneration of large motor neurons in the brain and spinal cord. 

Younger DS, Rowland LP, Latov N, Sherman W, PesceM, Lange DJ, etal. Motor neuron disease and amyotrophic lateral sclerosis Relation of high CSF protein content to paraproteinemia and clinical syndromes. Neurology 1990 40(4) 595-599. 

Amyotrophic lateral sclerosis (ALS) is one of the major forms of motor neuron disease (MND), a heterogeneous group of degenerative disorders causing progressive motor neuron death leading to paralysis and death. Amyotrophic lateral sclerosis is a relatively rare disease with a reported population incidence of between 1.5 and... 

Angelov DN, Waibel S, Gundnas-Lichius O, Lenzen M, Neiss WF, Tomov TL, Yoles E, Kipnis J, Schoii H, Reuter A, Ludolph A, Schwartz M (2003) Therapeutic vaccine for acute and chronic motor neuron diseases Implications for amyotrophic lateral sclerosis. ProcNatl Acad Sci 100 4790- 795. 

Amyotrophic lateral sclerosis is an inexorably progressive motor neuron disease, in w hich both the upper motor neurons and the lov er motor neurons degenerate leading to muscle atrophy. Patients eventually experience respiratory failure, usually within three to five years from diagnosis. However, the onset of AES may be subtle and early symptoms are frequendy overlooked. As many as 20,000 Americans have ALS, and an estimated 5,000 people in the United States are diagnosed with the disease each year. Onset is usually in the 5th through 7th decade of life. 

Orrell RW, Lane RJM, Ross M (2005) Andoxidant treatment for amyotrophic lateral sclerosis/motor neuron disease. The Cochrane Database for Systemadc Reviews, Volume 4, The Cochrane Col-labor adon. 

Appel SH, Engelhai dt JI, Gai cia J, Stefani E (1991) Immunoglobulins from animal models of motor neuron disease and from human amyod ophic lateral sclerosis padents passively ti ansfer physiological abnormalides to die neuromusculai juncdon. Proc Natl Acad SciUS A88 647-651. 

Miller RG, Mitchell JD, Moore DH (2002) Riluzole for amytophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev (2) CD001447. 

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