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Motor Neurone Disease Melanie Worthington Regional Care Development Adviser Lancashire & Cumbria Motor Neurone Disease Association Tel: 08453 751841 MND Connect: 08457 626262

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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

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Page 1: Neurological Facts

Motor Neurone Disease

Melanie WorthingtonRegional Care Development AdviserLancashire & CumbriaMotor Neurone Disease Association

Tel: 08453 751841

MND Connect: 08457 626262

Page 2: Neurological Facts

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

Page 3: Neurological Facts

What is Motor Neurone Disease

Page 4: Neurological Facts

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

Page 5: Neurological Facts

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

Page 6: Neurological Facts

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

Page 7: Neurological Facts

Causes of MND

Page 8: Neurological Facts

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

Page 9: Neurological Facts

Types of Motor Neurone Disease

Page 10: Neurological Facts

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

Page 11: Neurological Facts

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

Page 12: Neurological Facts

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

Page 13: Neurological Facts

Site of Onset

• Limb (usually distal)

• Bulbar

• Respiratory

Page 14: Neurological Facts

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

Page 15: Neurological Facts

Diagnosis of Motor Neurone Disease

Page 16: Neurological Facts

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

Page 17: Neurological Facts

How is MND Diagnosed?

• Interpretation of clinical symptoms and signs

• Investigations to exclude other causes

• MRI

• Lumbar puncture

• Lack of definitive test problematic

Page 18: Neurological Facts

Effects of Motor Neurone Disease

Page 19: Neurological Facts

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

Page 20: Neurological Facts

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

Page 21: Neurological Facts

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

Page 22: Neurological Facts

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)

Page 23: Neurological Facts

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

Page 24: Neurological Facts

Treatments and Interventions

Page 25: Neurological Facts

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

Page 26: Neurological Facts

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

Page 27: Neurological Facts

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).

Page 28: Neurological Facts

Multidisciplinary approach

Page 29: Neurological Facts

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

Page 30: Neurological Facts

MND Association Support

Page 31: Neurological Facts

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

Page 32: Neurological Facts

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]