Motor Neurone Disease Different types & Life Expectancy
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1 Motor Neurone Disease Different types & Life Expectancy S. ShabirGP HDR15th February 2011
2 What is MND? Progressive Degeneration of the motor neurones leads to: weakness and wasting of musclesloss of mobility in the limbsdifficulties with speech, swallowing and breathing.
3 MND Statistics Questions Who is affected more: Men women Equally Men are affected approximately twice as often as women.2) At what age does the highest incidence occurHow many people with MND at any one time in UK.There are about 5,000 people with MND at any one time in the UK.
4 Different types of MND Amyotrophic lateral sclerosis (ALS) This is the most common formAffects both upper and lower motor neurone involvement.Characterised by weakness and wasting in the limbsHand and arm symptoms.Reduced hand grip,drop things, difficult to open bottles, turn keysFeet and leg symptoms.Dragging of one leg, trip easily, difficulty to climb stairsMore tired after walkingAverage life expectancy is 2-5 years from onset of symptoms.
5 2. Progressive bulbar palsy (PBP) Affects about a quarter of people diagnosedInvolves both the upper and lower motor neuronesSymptoms: Slurring of speech or difficulty swallowingYou may not be able to shout or sing.Life expectancy is between six months and three years from onset of symptoms.
6 3. Progressive muscular atrophy (PMA) Affects only a small proportion of peopleMainly causing damage to the lower motor neuronesEarly symptoms may be noticed as weakness or clumsiness of the handMost people live for more than five years.
7 4. Primary lateral sclerosis (PLS) A rare form of MNDInvolving the upper motor neuronesCausing mainly weakness in the lower limbsSome people may experience clumsiness in the hands or speech problemsLife span could essentially be normalAlthough it may be life-limiting, depending on whether it remains as pure PLS or develops into ALS.
8 Respiratory failure is usually the cause of death. Eventually, the disease interferes with the muscles involved in breathing.Respiratory failure is usually the cause of death.
9 What is not affected in ALS-MND? Intellect does not usually changeHowever about 3% is associated with early dementia.Sensory parts of the nervous system are not affected, so you can feel, see, smell, taste and hear as before.Bladder and bowel function usually remains normal, so incontinence is not usually a feature. However, poor mobility may cause continence problems in the later stages of the illness.
10 Diagnostic Process Blood Tests A blood test will be looking to see if there is any rise in a creatine kinase.This is produced when muscle breaks down and can occasionally be found in the blood of people with MND.It is not specific for MND and may also be an indicator of other medical conditions.
11 Electromyography (EMG) The EMG test is sometimes called the needle test,because fine needles are used to record the naturally-occurring nerve impulses within certain muscles.Muscles, which have lost their nerve supply, can bedetected because their electrical activity is differentfrom normal healthy muscles.This is a very important diagnostic test.
12 Transcranial Magnetic Stimulation (TMS) This is a new procedure, which may be carried out at the same time as a nerve conduction test.It is designed to measure the activity of the upper motor neurones; its findings can help in the diagnostic process.