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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

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Neurological facts

Motor Neurone DiseaseMelanie WorthingtonRegional Care Development AdviserLancashire & CumbriaMotor Neurone Disease AssociationTel: 08453 751841MND Connect: 08457 626262


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



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


Who does it affect
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


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



Neurological facts

  • Familial – 5-10%

  • Rare

  • Research found genetic faults

  • SOD 1, FUS, VCP and TDP-43 genes

  • Ubiquilin protein gene

  • Chromosone 9

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



Neurological facts

  • Progressive Bulbar

  • Palsy (PBP)

  • 20% of cases (onset)

  • involves UMNs and

  • LMNs

  • dysarthria

  • dysphagia

  • emotional lability

  • progressive

  • weakness in upper

  • limbs/neck/

  • shoulder girdle

  • Amyotrophic Lateral

  • Sclerosis (ALS)

  • 65 - 66% of cases (onset)

  • involves UMNs and

  • LMNs

  • muscle weakness – often

  • develops in hands and

  • feet first, spasticity,

  • hyperactive reflexes


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 Lateral

  • Sclerosis (PLS)

  • 2% of cases

  • rare

  • UMNs only

  • muscle weakness

  • stiffness

  • balance

  • dysarthria

  • does not shorten

  • survival


Neurological facts

Courseof 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
Site of Onset

  • Limb (usually distal)

  • Bulbar

  • Respiratory


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



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


How is mnd diagnosed
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 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

Effects of MND


Clues to respiratory muscle involvement in mnd
Clues to respiratory muscle involvement in MND

  • Breathlessness

  • - on minimal exertion

  • - on lying flat

  • • Poor sleep

  • • Excessive daytime sleepiness

  • • Headaches on awakening

  • • Excessive nocturnalsweating


Psychosocial impact
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


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)


What isn t affected by mnd
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



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


Treatments interventions in mnd
Treatments/interventionsin MND

Multidisciplinary approach

Palliative care

Sensitive Management

Person with MND

Nutritional support

PEG/RIG

Rehabilitation medicine

Pharmaceutical management of symptoms

Respiratory care

Disease modifying therapy


Life prolonging interventions
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).



End of life decisions
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



Provided to plwmnd families carers and professionals
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
Motor Neurone Disease

  • Melanie WorthingtonRCDA Lancashire and CumbriaTel: 08453 751841 melanie.worthington@mndassociation.org

  • Preston MND Care & Research Centre

    Royal Preston HospitalTel: 01772 522545

  • MND Connect: 08457 626262

  • mndconnect@mndassociation.org