Download - Neurological Facts
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Motor Neurone Disease
Melanie WorthingtonRegional Care Development AdviserLancashire & CumbriaMotor Neurone Disease Association
Tel: 08453 751841
MND Connect: 08457 626262
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Neurological Facts• 10 million people in UK have a neurological condition
• Account for 20% of acute hospital admissions
• Third most common reason for visit to GP
• 850,000 carers
• 69% of primary care budget is spent on long term conditions(Department of Health)
• MND – over 5,000 people in UK
• Parkinson’s Disease – 120,000
• Multiple Sclerosis – 100,000
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What is Motor Neurone Disease
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Motor Neurone Disease
• Every person develops the disease in a different way
• Symptoms experienced depends on the area of nervous system affected
• 90% - 95% of people have the sporadic form (out of the blue)
• 5-10% Familial – 200-300 people
• Adult Illness – most people are over 50
• Average survival 2-5 years from first symptoms.
• From diagnosis 14 months average.
• No cure but symptom management and medication that may improve quality or prolong life
• Onset and progression is variable – can progress swiftly
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Who does it affect ?
• Relatively uncommon• Annual incidence of 2 in 100,000• Prevalence 5-7 per 100,000• More common in men but over 65 yrs
becomes more even• GPs can expect to see 1 or 2 cases during
their career
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What is Motor Neurone Disease?• Upper motor neurones (UMN)
originate in the base of the cortex of the brain : Spasticity
• Lower motor neurones (LMN) originate in the spinal cord: Wasting/Weakness
• Act as transmitters that provide a chain of command for voluntary movement to muscles throughout the body
• In MND this chain of command is broken as neurones degenerate
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Causes of MND
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Sporadic – 90%
• Risk factors: genetic, environmental and lifestyle factors that may tip the balance:
- mechanical/electrical trauma- Military service- High levels of exercise- Agricultural chemicals and
heavy metals
Evidence is often circumstantial
and conflicting
Familial – 5-10%
•Rare•Research found genetic faults•SOD 1, FUS, VCP and TDP-43 genes•Ubiquilin protein gene•Chromosone 9
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Types of Motor Neurone Disease
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Amyotrophic LateralSclerosis (ALS)
65 - 66% of cases (onset)
• involves UMNs and LMNs• muscle weakness – often• develops in hands and • feet first, spasticity,• hyperactive reflexes
Progressive BulbarPalsy (PBP)20% of cases (onset)
•involves UMNs and LMNs• dysarthria• dysphagia • emotional lability• progressive weakness in upper limbs/neck/ shoulder girdle
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Progressive Muscular Atrophy (PMA)7.5% - 10% of cases
• predominantly LMNs affected (may start in small muscles of hand)• muscle wasting, weakness• fasciculation(may in time develop UMN involvement and may eventually develop some speech problems)
Primary LateralSclerosis (PLS)2% of cases
• rare• UMNs only• muscle weakness• stiffness• balance• dysarthria• does not shorten survival
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Course of Disease
• Onset and progression variable
• Is always progressive with no remissions
• Usually affects both the upper and lower motor neurones
• 90% develop some bulbar symptoms
• Death often through respiratory failure
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Site of Onset
• Limb (usually distal)
• Bulbar
• Respiratory
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Early Symptoms
Depend on area of nervous system affected:
• stumbling• foot drop• loss of dexterity• weakened grip• cramps• change of voice quality• slurred speech• early swallowing difficulties• muscle wasting • fatigue
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Diagnosis of Motor Neurone Disease
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Diagnosis
• On average, it takes 14 months from first symptoms to diagnose MND
• First signs and symptoms often subtle and non-specific, similar to other diseases
• Person often not referred to a neurologist directly
• No definitive diagnostic test
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How is MND Diagnosed?
• Interpretation of clinical symptoms and signs
• Investigations to exclude other causes
• MRI
• Lumbar puncture
• Lack of definitive test problematic
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Effects of Motor Neurone Disease
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Effects of MND
• Progressive muscle weakness and wasting
• Loss of weight• Fasciculation, cramp
and spasticity• Dysarthria-slurred
effortful speech• Saliva and Mucus
Problems
• Dysphagia - poor swallow due to weakness and paralysis of bulbar muscles
• Respiratory muscle weakness
• emotional lability• Cognitive changes
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Clues to respiratory muscle involvement in MND
•Breathlessness - on minimal exertion - on lying flat• Poor sleep• Excessive daytime sleepiness• Headaches on awakening• Excessive nocturnal sweating
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Psychosocial Impact
• Multiple losses: physical loss, loss of control, role, independence, self image, self esteem and confidence
• Financial • Home environment• Communication difficulties• Increasing isolation and dependence on carers• Anxiety, Fear, Anger• Knowledge of own impending deterioration and death
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Cognitive changes
• MND has been traditionally viewed at a disease affecting the motor system with no compromise of cognitive abilities• Recent research shows that 25% or more show some cognitive changes in the frontal lobe region• 3-5% will have fronto-temporal dementia (FTD)
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What isn’t affected by MND
• Senses: touch, taste, sight, smell and hearing
• Bowel and bladder function
• Sexual function and sexuality
• Eye Muscles
• Heart muscles
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Treatments and Interventions
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Aims of Management
•Control of symptoms • Promote independence and control – usually supported at home as much as possible
• Plan appropriate interventions
• Enable person with MND and family to live as full a life as possible
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Treatments/interventions in MNDMultidisciplinary
approach
Sensitive Management
Nutritional support
PEG/RIG
Respiratory care
Disease modifying therapy
Pharmaceutical management of
symptoms
Rehabilitation medicine
Palliative care
Person with MND
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Life Prolonging Interventions
• Riluzole only drug to have beneficial effect on survival : 3-4 months
• Respiratory care: Non-invasive ventilation (NIV)
• To improve quality of life.• Median survival extended 205
days (Miller et all 2009).
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Multidisciplinary approach
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End of Life Decisions
• Advanced Care Planning• Advanced decision to refuse treatment
(ADART)• Advanced Statment of wishes and
preferences• Preferred Priorities of Care (PPC)• Withdrawal of treatments• Tissue donations
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MND Association Support
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Provided to plwMND, families, carers and professionals
• Standards of Care
• Regional Care Development Advisers
• Association Visitors and Volunteers
• Equipment Loan
• Financial Support
• Care Information
• MND Connect
• Local Branch Network
• Care Centre Programme
• Education/Training
• www.mndassociation.org
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Motor Neurone Disease• Melanie Worthington
RCDA Lancashire and CumbriaTel: 08453 751841 [email protected]
• Preston MND Care & Research Centre Royal Preston Hospital
Tel: 01772 522545
• MND Connect: 08457 626262• [email protected]