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The motor neuron diseases are a group of progressive neurological disorders that destroy motor neurons, the cells that control essential voluntary muscle activity such as speaking, walking, breathing, and swallowing. Normally, messages from nerve cells in the brain are transmitted to nerve cells in the brain stem and spinal cord and from them to particular muscles. Upper motor neurons direct the lower motor neurons to produce movements such as walking or chewing. Lower motor neurons control movement in the arms, legs, chest, face, throat, and tongue. Some of the more common MNDs are as follows amyotrophic lateral sclerosis, Progressive bulbar palsy, Pseudobulbar palsy, Primary lateral sclerosis and Spinal muscular atrophy.
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Motor neuron diseases can affect any adult at any age but most people diagnosed with the disease are over the age of 40, with the highest incidence occurring between the ages of 50 and 70. Men are affected approximately twice as often as women. MND affects 4-6 people out of every 100 000. MND usually presents in middle age and is slightly more common in men. In New Zealand about 300 people have motor neurone disease at any one time. Slightly more men than women develop the disease. An estimated 1,900 people have MND in Australia, for every person diagnosed with MND it is estimated that a further 14 members of their family and their friends will live with the effect of MND forever. In 2011, 790 people with MND died compared with 592 people with MND who died in 2001. The cause of this increase is mostly unknown.
The global market for neurological disease treatment and medication was worth $12.6 billion in 2006 and will reach $14 billion by 2007. At a compound annual growth rate (CAGR) of 11.6%, the global market will be worth almost $24.3 billion by 2012. Owing to the high-impact growth drivers that outweigh the restraints, the global neuro market is forecast to reach $14 billion by 2020, registering a CAGR of 15.8% from 2014 to 2020.
Motor neuron diseases are a fatal neurodegenerative disease. It is characterized by the onset of symptoms and signs of degeneration of primarily the upper and lower motor neurones. This leads to progressive weakness of the bulbar, limb, thoracic and abdominal muscles.
Respiratory muscle weakness resulting in respiratory impairment is a major feature of MND, and is a strong predictor of quality of life and survival. Non-invasive ventilation can improve symptoms and signs related to respiratory impairment and hence survival.
There is currently no evidence-based guideline for use in England, Wales and Northern Ireland that addresses the use of non-invasive ventilation in patients with MND. This guideline considers the signs and symptoms that can be used for predicting respiratory impairment in patients with MND, the diagnostic accuracy of investigations for detecting and monitoring respiratory impairment, the clinical and cost effectiveness of non-invasive ventilation for treating respiratory impairment and the information and support needs of patients and their families and carers relating to the use of non-invasive ventilation.
The motor neuron diseases (MNDs) are a group of progressive neurological disordersthat destroys motor neurons, the cells that control essential voluntary muscle activity such as speaking, walking, breathing, and swallowing.
Some MNDs are inherited, but the causes of most MNDs are not known. In sporadic or non inherited MNDs, environmental, toxic, viral, or genetic factors may be implicated. There are no specific tests to diagnose most MNDs although there are now gene tests for SMA. Symptoms may vary among individuals and, in the early stages of the disease, may be similar to those of other diseases, making diagnosis difficult.
Electromyography (EMG) is used to diagnose disorders of lower motor neurons, as well as disorders of muscle and peripheral nerves. There is no cure or standard treatment for the MNDs. Symptomatic and supportive treatment can help people be more comfortable while maintaining their quality of life.
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This page was last updated on June 2, 2023