Upload
charity-stanton
View
42
Download
0
Embed Size (px)
DESCRIPTION
Motor Neurone Disease Melanie Worthington Regional Care Development Adviser Lancashire & Cumbria Motor Neurone Disease Association Tel: 08453 751841 MND Connect: 08457 626262. Neurological Facts. 10 million people in UK have a neurological condition Account for 20% of acute hospital admissions - PowerPoint PPT Presentation
Citation preview
Motor Neurone Disease
Melanie WorthingtonRegional Care Development AdviserLancashire & CumbriaMotor Neurone Disease Association
Tel: 08453 751841
MND Connect: 08457 626262
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
What is Motor Neurone Disease
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
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
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
Causes of MND
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
Types of Motor Neurone Disease
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
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
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
Site of Onset
• Limb (usually distal)
• Bulbar
• Respiratory
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
Diagnosis of Motor Neurone Disease
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
How is MND Diagnosed?
• Interpretation of clinical symptoms and signs
• Investigations to exclude other causes
• MRI
• Lumbar puncture
• Lack of definitive test problematic
Effects of Motor Neurone Disease
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
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
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
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)
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
Treatments and Interventions
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
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
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).
Multidisciplinary approach
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
MND Association Support
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
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]