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Motor neurone disease

What is Motor Neurone Disease (MND)

The term "MND" actually refers to a group of diseases (Classification of Motor Neurone Diseases), the most common of which is Amyotrophic Lateral Sclerosis (ALS), or "Lou Gehrig's Disease". This affects approximately 85% of patients diagnosed with MND and in the UK, approximately 5,000 people are affected at any one time. It is characterised by the progressive loss of motor neurones in the brain and spinal cord. These carry signals from the brain to the muscles themseles, telling them when to contract and when to relax. MND only affects the skeletal muscles, that is, muscles which control the limbs, face, and torso. The internal muscles of the body (such as the heart, smooth muscle of the digestive tract, and sphincters) are unaffected. In the vast majority of cases, the neurones controlling eye movements are also unaffected. The disease is inevitably fatal, although the course of the disease can vary significantly. The average survival from diagnosis is quoted as being between 2-5 years, with 50% of patients dying in the first 14 months. Approximately 1 in 5 patients lives beyond 5 years, and 1 in 10 patients lives beyond 10 years.

The disease involves degeneration of both the upper motor neurones (UMNs), which originate in the brain and travel down the spinal cord, and the lower motor neurones (LMNs) which go from the muscles themselves to the spinal cord, where they meet (or "synapse") with the UMNs (Pathological features of ALS). Two rarer forms of MND affect only one set or the other. Primary Lateral Sclerosis (PLS) affects only the UMNs, sparing the spinal LMNs. PLS affects about 10% of people diagnosed with MND and has a much longer survival time, giving patients an effectively normal life span. However, they still suffer from a progressive and debilitating chronic illness and require a more prolonged period of care. Progressive Muscular Atrophy (PMA) affects approximately 5% of MND patients and affects only the spinal LMNs. Like PLS, PMA is associated with a better prognosis than ALS (approximately 5-10 years), but is still fatal.

The most well known living person with MND is Professor Stephen Hawking, who says in his introduction to "A Brief History of Time" that he suffers from ALS rather than one of the rarer subtypes of MND. Having first acquired the disease in his early twenties, Professor Hawking is one of the longest recorded sufferers of the disease. Other well known people that have suffered from MND are the actor David Niven, the Celtic footballer Jimmy Johnstone, and the American baseball player Lou Gehrig, a contemporary of Babe Ruth from the 1930's.

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